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Hirano M, Tsujie M, Goto T, Koga C, Mori S, Takiuchi D, Nishida K, Nomura M, Yoshikawa Y, Tamai K, Hamakawa T, Tei M, Akamaru Y. Spontaneously Ruptured Pancreatic Mucinous Cystic Neoplasm: A Case Report. Surg Case Rep 2025; 11:24-0087. [PMID: 40008371 PMCID: PMC11850215 DOI: 10.70352/scrj.cr.24-0087] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/22/2024] [Accepted: 12/31/2024] [Indexed: 02/27/2025] Open
Abstract
INTRODUCTION Pancreatic mucinous cystic neoplasm (MCN) is a cystic tumor of the pancreas typically located in the pancreatic body or tail in middle-aged women. However, MCN rupture is rare. This report describes a case of MCN with spontaneous rupture during follow-up. CASE PRESENTATION The patient was a 34-year-old woman. Contrast-enhanced computed tomography (CECT) and magnetic resonance imaging (MRI) revealed a 130 mm multifocal cyst in the pancreatic tail. The cyst, characterized by multiple septa and cyst-in-cyst structures, was diagnosed as an MCN. Initially, the patient opted for periodic follow-ups instead of surgical resection. After a gradual increase in cyst size, surgery was scheduled approximately 1 year later. Two days before the scheduled surgery, the patient experienced unexplained lower abdominal pain. Moreover, CECT revealed a shrinking cystic mass in the pancreatic tail along with the presence of ascites, leading to a diagnosis of spontaneous rupture of the pancreatic cyst. No peritonitis was detected, and a distal pancreatectomy was performed 2 days after admission. Pathological examination confirmed that the pancreatic cyst was a noninvasive mucinous cystadenocarcinoma. The abdominal cavity contained large amounts of turbid ascites with neutrophils but no bacterial growth. Strong inflammatory changes were noted at the cyst wall disruption site. Despite the development of a pancreatic fistula (ISGPF Grade BL, Clavien-Dindo Grade II), the patient was discharged from the hospital on postoperative day 16 and remained alive and recurrence-free for 18 months after surgery. CONCLUSION Spontaneous rupture of an MCN is rare. In this study, we report our case and review previously published cases of MCN rupture. We also discuss the potential causes of the spontaneous rupture in our case.
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Affiliation(s)
- Masataka Hirano
- Department of Gastroenterological Surgery, Osaka Rosai Hospital, Sakai, Osaka, Japan
| | - Masanori Tsujie
- Department of Gastroenterological Surgery, Osaka Rosai Hospital, Sakai, Osaka, Japan
| | - Takayoshi Goto
- Department of Diagnostic Pathology, Osaka Rosai Hospital, Sakai, Osaka, Japan
| | - Chikato Koga
- Department of Gastroenterological Surgery, Osaka Rosai Hospital, Sakai, Osaka, Japan
| | - Soichiro Mori
- Department of Gastroenterological Surgery, Osaka Rosai Hospital, Sakai, Osaka, Japan
| | - Daisuke Takiuchi
- Department of Gastroenterological Surgery, Osaka Rosai Hospital, Sakai, Osaka, Japan
| | - Kentaro Nishida
- Department of Gastroenterological Surgery, Osaka Rosai Hospital, Sakai, Osaka, Japan
| | - Masatoshi Nomura
- Department of Gastroenterological Surgery, Osaka Rosai Hospital, Sakai, Osaka, Japan
| | - Yukihiro Yoshikawa
- Department of Gastroenterological Surgery, Osaka Rosai Hospital, Sakai, Osaka, Japan
| | - Koki Tamai
- Department of Gastroenterological Surgery, Osaka Rosai Hospital, Sakai, Osaka, Japan
| | - Takuya Hamakawa
- Department of Gastroenterological Surgery, Osaka Rosai Hospital, Sakai, Osaka, Japan
| | - Mitsuyoshi Tei
- Department of Gastroenterological Surgery, Osaka Rosai Hospital, Sakai, Osaka, Japan
| | - Yusuke Akamaru
- Department of Gastroenterological Surgery, Osaka Rosai Hospital, Sakai, Osaka, Japan
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De Stefano F, Pellegrini R, Marchegiani G, Crippa S. Reducing the burden of pancreatic cancer by surveilling mucinous cystic neoplasms: are we there yet? Best Pract Res Clin Gastroenterol 2025; 74:101998. [PMID: 40210333 DOI: 10.1016/j.bpg.2025.101998] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/18/2024] [Revised: 02/27/2025] [Accepted: 03/10/2025] [Indexed: 04/12/2025]
Abstract
Pancreatic cystic neoplasms are a common condition, with intraductal papillary mucinous neoplasms (IPMNs) and mucinous cystic neoplasms (MCNs) being of particular concern due to their potential for malignant transformation. Resection of these lesions before progression to invasive cancer represents a crucial opportunity to reduce the burden of pancreatic cancer. However, the benefit of early-resection must be weighed against the risk of overtreatment. Applying current guidelines, nearly half of the patients who undergo resection for IPMN might not have required surgery, while malignancy is detected in only 16 % of resected MCNs. This raises concerns, particularly given the increasing detection of small cysts with the widespread use of radiological imaging and the significant postoperative risks, including exocrine and endocrine insufficiency. Emerging evidence suggests that surveillance strategies could be more broadly applied, reducing unnecessary surgeries. This review aims to reassess the current evidence regarding both surgical resection and surveillance of IPMNs and MCNs.
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Affiliation(s)
- Federico De Stefano
- Division of Pancreatic Surgery, Pancreas Translational & Clinical Research Center, IRCCS San Raffaele Scientific Institute, Milan, Italy; Vita-Salute San Raffaele University, Milan, Italy
| | - Riccardo Pellegrini
- Department of Surgical Oncological and Gastroenterological Sciences, Padua University Hospital, Padua, Italy
| | - Giovanni Marchegiani
- Department of Surgical Oncological and Gastroenterological Sciences, Padua University Hospital, Padua, Italy.
| | - Stefano Crippa
- Division of Pancreatic Surgery, Pancreas Translational & Clinical Research Center, IRCCS San Raffaele Scientific Institute, Milan, Italy; Vita-Salute San Raffaele University, Milan, Italy
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Seyithanoglu D, Durak G, Keles E, Medetalibeyoglu A, Hong Z, Zhang Z, Taktak YB, Cebeci T, Tiwari P, Velichko YS, Yazici C, Tirkes T, Miller FH, Keswani RN, Spampinato C, Wallace MB, Bagci U. Advances for Managing Pancreatic Cystic Lesions: Integrating Imaging and AI Innovations. Cancers (Basel) 2024; 16:4268. [PMID: 39766167 PMCID: PMC11674829 DOI: 10.3390/cancers16244268] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/24/2024] [Revised: 12/08/2024] [Accepted: 12/19/2024] [Indexed: 01/11/2025] Open
Abstract
Pancreatic cystic lesions (PCLs) represent a spectrum of non-neoplasms and neoplasms with varying malignant potential, posing significant challenges in diagnosis and management. While some PCLs are precursors to pancreatic cancer, others remain benign, necessitating accurate differentiation for optimal patient care. Conventional approaches to PCL management rely heavily on radiographic imaging, and endoscopic ultrasound (EUS) guided fine-needle aspiration (FNA), coupled with clinical and biochemical data. However, the observer-dependent nature of image interpretation and the complex morphology of PCLs can lead to diagnostic uncertainty and variability in patient management strategies. This review critically evaluates current PCL diagnosis and surveillance practices, showing features of the different lesions and highlighting the potential limitations of conventional methods. We then explore the potential of artificial intelligence (AI) to transform PCL management. AI-driven strategies, including deep learning algorithms for automated pancreas and lesion segmentation, and radiomics for analyzing heterogeneity, can improve diagnostic accuracy and risk stratification. These advanced techniques can provide more objective and reproducible assessments, aiding clinicians in decision-making regarding follow-up intervals and surgical interventions. Early results suggest that AI-driven methods can significantly improve patient outcomes by enabling earlier detection of high-risk lesions and reducing unnecessary procedures for benign cysts. Finally, this review emphasizes that AI-driven approaches could potentially reshape the landscape of PCL management, ultimately leading to improved pancreatic cancer prevention.
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Affiliation(s)
- Deniz Seyithanoglu
- Machine and Hybrid Intelligence Lab, Feinberg School of Medicine, Northwestern University, Chicago, IL 60611, USA; (D.S.); (G.D.); (E.K.); (A.M.); (Z.H.); (Z.Z.); (Y.S.V.); (F.H.M.); (R.N.K.)
- Istanbul Faculty of Medicine, Istanbul University, Istanbul 38000, Turkey; (Y.B.T.); (T.C.)
| | - Gorkem Durak
- Machine and Hybrid Intelligence Lab, Feinberg School of Medicine, Northwestern University, Chicago, IL 60611, USA; (D.S.); (G.D.); (E.K.); (A.M.); (Z.H.); (Z.Z.); (Y.S.V.); (F.H.M.); (R.N.K.)
| | - Elif Keles
- Machine and Hybrid Intelligence Lab, Feinberg School of Medicine, Northwestern University, Chicago, IL 60611, USA; (D.S.); (G.D.); (E.K.); (A.M.); (Z.H.); (Z.Z.); (Y.S.V.); (F.H.M.); (R.N.K.)
| | - Alpay Medetalibeyoglu
- Machine and Hybrid Intelligence Lab, Feinberg School of Medicine, Northwestern University, Chicago, IL 60611, USA; (D.S.); (G.D.); (E.K.); (A.M.); (Z.H.); (Z.Z.); (Y.S.V.); (F.H.M.); (R.N.K.)
- Istanbul Faculty of Medicine, Istanbul University, Istanbul 38000, Turkey; (Y.B.T.); (T.C.)
| | - Ziliang Hong
- Machine and Hybrid Intelligence Lab, Feinberg School of Medicine, Northwestern University, Chicago, IL 60611, USA; (D.S.); (G.D.); (E.K.); (A.M.); (Z.H.); (Z.Z.); (Y.S.V.); (F.H.M.); (R.N.K.)
| | - Zheyuan Zhang
- Machine and Hybrid Intelligence Lab, Feinberg School of Medicine, Northwestern University, Chicago, IL 60611, USA; (D.S.); (G.D.); (E.K.); (A.M.); (Z.H.); (Z.Z.); (Y.S.V.); (F.H.M.); (R.N.K.)
| | - Yavuz B. Taktak
- Istanbul Faculty of Medicine, Istanbul University, Istanbul 38000, Turkey; (Y.B.T.); (T.C.)
| | - Timurhan Cebeci
- Istanbul Faculty of Medicine, Istanbul University, Istanbul 38000, Turkey; (Y.B.T.); (T.C.)
| | - Pallavi Tiwari
- Department of Radiology, BME, University of Wisconsin-Madison, Madison, WI 53707, USA;
- William S. Middleton Memorial Veterans Affairs (VA) Healthcare, 2500 Overlook Terrace, Madison, WI 53705, USA
| | - Yuri S. Velichko
- Machine and Hybrid Intelligence Lab, Feinberg School of Medicine, Northwestern University, Chicago, IL 60611, USA; (D.S.); (G.D.); (E.K.); (A.M.); (Z.H.); (Z.Z.); (Y.S.V.); (F.H.M.); (R.N.K.)
| | - Cemal Yazici
- Department of Gastroenterology, University of Illinois at Chicago, Chicago, IL 60611, USA;
| | - Temel Tirkes
- Department of Radiology, Indiana University, Indianapolis, IN 46202, USA;
| | - Frank H. Miller
- Machine and Hybrid Intelligence Lab, Feinberg School of Medicine, Northwestern University, Chicago, IL 60611, USA; (D.S.); (G.D.); (E.K.); (A.M.); (Z.H.); (Z.Z.); (Y.S.V.); (F.H.M.); (R.N.K.)
| | - Rajesh N. Keswani
- Machine and Hybrid Intelligence Lab, Feinberg School of Medicine, Northwestern University, Chicago, IL 60611, USA; (D.S.); (G.D.); (E.K.); (A.M.); (Z.H.); (Z.Z.); (Y.S.V.); (F.H.M.); (R.N.K.)
| | - Concetto Spampinato
- Department of Electrical, Electronics and Computer Engineering, University of Catania, 95124 Catania, Italy;
| | - Michael B. Wallace
- Department of Gastroenterology, Mayo Clinic Florida, Jacksonville, FL 32224, USA;
| | - Ulas Bagci
- Machine and Hybrid Intelligence Lab, Feinberg School of Medicine, Northwestern University, Chicago, IL 60611, USA; (D.S.); (G.D.); (E.K.); (A.M.); (Z.H.); (Z.Z.); (Y.S.V.); (F.H.M.); (R.N.K.)
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Sung YN, Stojanova M, Shin S, Cho H, Heaphy CM, Hong SM. Gradual telomere shortening in the tumorigenesis of pancreatic and hepatic mucinous cystic neoplasms. Hum Pathol 2024; 152:105653. [PMID: 39214240 DOI: 10.1016/j.humpath.2024.105653] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/11/2024] [Revised: 08/22/2024] [Accepted: 08/27/2024] [Indexed: 09/04/2024]
Abstract
Mucinous cystic neoplasm (MCN) is one of the precursor lesions of pancreatic ductal adenocarcinoma and intrahepatic cholangiocarcinoma. The aim of this study is to examine the presence of short telomeres in promoting the tumorigenesis of MCN by measuring telomere lengths in distinct components of MCN, including the mucinous lining epithelium, non-mucinous lining epithelium, and ovarian-type stroma. A total of 45 patients with MCN (30 pancreatic and 15 hepatic cases) were obtained. Quantitative telomere-specific fluorescent in situ hybridization was performed to measure the telomere length of specific cell types within MCNs, including mucinous lining epithelium, non-mucinous lining epithelium, and ovarian-type stroma, as well as normal ductal epithelium and adenocarcinoma. Relative telomere lengths tended to decrease between normal ductal epithelium, ovarian-type stroma, non-mucinous lining epithelium, mucinous lining epithelium, and adenocarcinoma regardless of the involved organs. Among the analyzed cell types, relative telomere lengths were significantly different between normal ductal epithelium (3.31 ± 0.78), ovarian-type stroma (2.90 ± 0.93), non-mucinous lining epithelium (2.84 ± 0.79), mucinous lining epithelium (2.49 ± 0.93), and adenocarcinoma (1.19 ± 0.59), respectively (P < 0.001, mixed-effects model). As expected, no difference in relative telomere lengths was observed between normal ductal epithelium and ovarian-type stroma; however, significant differences were observed in pair-wise comparisons between ovarian-type stroma vs. non-mucinous lining epithelium (P = 0.001), non-mucinous lining epithelium vs. mucinous lining epithelium (P = 0.005), and mucinous lining epithelium vs. adenocarcinoma (P < 0.001). These findings suggest gradual telomere shortening occurs in the tumorigenesis of MCN, which may have important implications for the progression of this disease.
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Affiliation(s)
- You-Na Sung
- Department of Pathology, Korea University Anam Hospital, Korea University College of Medicine, Seoul, Republic of Korea
| | - Marija Stojanova
- Department of Medicine, Boston University Chobanian & Avedisian School of Medicine, Boston, MA, USA
| | - Seungbeom Shin
- Department of Statistics, Korea University, Seoul, Republic of Korea
| | - HyungJun Cho
- Department of Statistics, Korea University, Seoul, Republic of Korea
| | - Christopher M Heaphy
- Department of Medicine, Boston University Chobanian & Avedisian School of Medicine, Boston, MA, USA; Department of Pathology and Laboratory Medicine, Boston University Chobanian & Avedisian School of Medicine, Boston, MA, USA.
| | - Seung-Mo Hong
- Department of Pathology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Republic of Korea.
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Rogowska J, Semeradt J, Durko Ł, Małecka-Wojciesko E. Diagnostics and Management of Pancreatic Cystic Lesions-New Techniques and Guidelines. J Clin Med 2024; 13:4644. [PMID: 39200786 PMCID: PMC11355509 DOI: 10.3390/jcm13164644] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/08/2024] [Revised: 08/01/2024] [Accepted: 08/06/2024] [Indexed: 09/02/2024] Open
Abstract
Pancreatic cystic lesions (PCLs) are increasingly diagnosed owing to the wide use of cross-sectional imaging techniques. Accurate identification of PCL categories is critical for determining the indications for surgical intervention or surveillance. The classification and management of PCLs rely on a comprehensive and interdisciplinary evaluation, integrating clinical data, imaging findings, and cyst fluid markers. EUS (endoscopic ultrasound) has become the widely used diagnostic tool for the differentiation of pancreatic cystic lesions, offering detailed evaluation of even small pancreatic lesions with high sensitivity and specificity. Additionally, endoscopic ultrasound-fine-needle aspiration enhances diagnostic capabilities through cytological analysis and the assessment of fluid viscosity, tumor glycoprotein concentration, amylase levels, and molecular scrutiny. These detailed insights play a pivotal role in improving the clinical prognosis and management of pancreatic neoplasms. This review will focus mainly on the latest recommendations for the differentiation, management, and treatment of pancreatic cystic lesions, highlighting their clinical significance.
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Affiliation(s)
- Jagoda Rogowska
- Department of Digestive Tract Diseases, Medical University of Lodz, 90-647 Lodz, Poland; (J.S.); (Ł.D.); (E.M.-W.)
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Moriuchi T, Hashimoto M, Kuroda S, Kobayashi T, Ohdan H. Mucinous Cystic Neoplasm of the Liver in a Teenager: A Case Report. Cureus 2024; 16:e65728. [PMID: 39211699 PMCID: PMC11359318 DOI: 10.7759/cureus.65728] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 07/29/2024] [Indexed: 09/04/2024] Open
Abstract
Mucinous cystic neoplasms of the liver (MCN-Ls) are rare cystic liver tumors. Herein, we report a case of MCN-L wherein complete surgical resection was successful. A 17-year-old girl initially presented to the referring hospital with a chief complaint of upper abdominal pain. Abdominal computed tomography (CT) revealed a cystic lesion in the medial segment of the liver. After eight months, the cystic lesion showed a tendency to increase in size, and the patient was referred to our hospital. CT showed a cystic lesion with dilation of the left hepatic duct and duct of the right anterior segment. Magnetic resonance imaging and abdominal ultrasonography revealed a multilocular cyst. Endoscopic retrograde cholangiopancreatography revealed sclerotic changes, dilatation, and irregular wall features in the left hepatic duct. No communication between the cystic lesion and the biliary system was observed; there was no evidence of biliary prolapse. A left hepatectomy and cholecystectomy were performed. Histological examination revealed an ovarian-like stroma (OLS); the lesion was diagnosed as MCN-L. The patient was recurrence-free six months postoperatively.To our best knowledge, this is the second reported case of teenage-onset MCN-L. We report the development of MCN-L in a teenager, highlighting the potential of this rare tumor for manifesting even at a young age. Our case demonstrated that MCN-L, despite its typically benign nature, should be carefully monitored. Although most cases of MCN-L do not require immediate surgery, timely surgical intervention may be necessary in cases of rapid growth or persistent symptoms.
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Affiliation(s)
- Toshiyuki Moriuchi
- Department of Gastroenterological and Transplant Surgery, Graduate School of Biomedical and Health Science, Hiroshima University, Hiroshima, JPN
| | - Masakazu Hashimoto
- Department of Gastroenterological and Transplant Surgery, Graduate School of Biomedical and Health Science, Hiroshima University, Hiroshima, JPN
| | - Shintaro Kuroda
- Department of Gastroenterological and Transplant Surgery, Graduate School of Biomedical and Health Science, Hiroshima University, Hiroshima, JPN
| | - Tsuyoshi Kobayashi
- Department of Gastroenterological and Transplant Surgery, Graduate School of Biomedical and Health Science, Hiroshima University, Hiroshima, JPN
| | - Hideki Ohdan
- Department of Gastroenterological and Transplant Surgery, Graduate School of Biomedical and Health Science, Hiroshima University, Hiroshima, JPN
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Scheyda-Yoo K, Hofer U, Várnai-Händel A, Maus MKH, Dumoulin FL. [Rapid growth and malignant transformation of a mucinous cystic neoplasm during pregnancy - a case report]. ZEITSCHRIFT FUR GASTROENTEROLOGIE 2024; 62:1048-1052. [PMID: 38657617 DOI: 10.1055/a-2239-7898] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 04/26/2024]
Abstract
BACKGROUND Mucinous-cystic neoplasms (MCN) account for 10% of all pancreatic cystic lesions. They are found almost exclusively in females. MCN have an ovarian-like stroma and often estrogen and progesterone receptors. During pregnancy, they can massively increase in size and transform into malignancy. CASE REPORT We report on a 29-year-old woman in whom a 35mm cyst in the pancreatic tail had been diagnosed several years ago. After workup the lesions had been classified as a pseudocyst. During pregnancy, the cyst massively increased in size and finally was resected. Histology showed a mucinous-cystic neoplasia with focal malignant transformation. CONCLUSION Cystic neoplasms of the pancreas require a differentiated management. While overtreatment should be avoided, malignant transformation always merits consideration - in particular if the cystic lesion is located in the pancreatic tail. Women with suspected MCN or cystic pancreatic lesions of uncertain etiology should be informed about the (rare) risk of a malignant transformation of an MCN and should be closely monitored during pregnancy.
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Affiliation(s)
- Katharina Scheyda-Yoo
- Abteilung für Innere Medizin, Gemeinschaftskrankenhaus St Elisabeth St Petrus St Johannes gGmbH, Bonn, Germany
| | - Ulrich Hofer
- Abteilung für Radiologie, Interventionelle Radiologie und Neuroradiologie, Gemeinschaftskrankenhaus St Elisabeth St Petrus St Johannes gGmbH, Bonn, Germany
| | | | - Martin K-H Maus
- Funktionelle OGI Chirurgie, Evangelisches Krankenhaus Kalk, Koln, Germany
| | - Franz Ludwig Dumoulin
- Abteilung für Innere Medizin, Gemeinschaftskrankenhaus St Elisabeth St Petrus St Johannes gGmbH, Bonn, Germany
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Thorner PS, Chong AL, Apellaniz-Ruiz M, Benlimame N, Marrano P, Brimo F, Shuangshoti S, Shuangshoti S, Foulkes WD. Estrogen Receptor Expression in DICER1-related Lesions is Associated With the Presence of Cystic Components. Am J Surg Pathol 2024; 48:733-741. [PMID: 38539053 DOI: 10.1097/pas.0000000000002209] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 05/16/2024]
Abstract
DICER1 tumor predisposition syndrome results from pathogenic variants in DICER1 and is associated with a variety of benign and malignant lesions, typically involving kidney, lung, and female reproductive system. Over 70% of sarcomas in DICER1 tumor predisposition syndrome occur in females. Notably, pediatric cystic nephroma (pCN), a classic DICER1 tumor predisposition syndrome lesion, shows estrogen receptor (ER) expression in stromal cells. There are also renal, hepatic, and pancreatic lesions unassociated with DICER1 tumor predisposition syndrome that have an adult female predominance and are characterized/defined by ER-positive stromal cells. Except for pCN, the expression of ER in DICER1-associated lesions remains uninvestigated. In the present study, ER expression was assessed by immunohistochemistry in 89 cases of DICER1-related lesions and 44 lesions lacking DICER1 pathogenic variants. Expression was seen in stromal cells in pCN and pleuropulmonary blastoma (PPB) types I and Ir, whereas anaplastic sarcoma of kidney and PPB types II and III were typically negative, as were other solid tumors of non-Müllerian origin. ER expression was unrelated to the sex or age of the patient. Expression of ER showed an inverse relationship to preferentially expressed antigen in melanoma (PRAME) expression; as lesions progressed from cystic to solid (pCN/anaplastic sarcoma of kidney, and PPB types I to III), ER expression was lost and (PRAME) expression increased. Thus, in DICER1 tumor predisposition syndrome, there is no evidence that non-Müllerian tumors are hormonally driven and antiestrogen therapy is not predicted to be beneficial. Lesions not associated with DICER1 pathogenic variants also showed ER-positive stromal cells, including cystic pulmonary airway malformations, cystic renal dysplasia, and simple renal cysts in adult kidneys. ER expression in stromal cells is not a feature of DICER1 perturbation but rather is related to the presence of cystic components.
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Affiliation(s)
- Paul Scott Thorner
- Department of Laboratory Medicine and Pathobiology, University of Toronto, Toronto, Canada
| | - Anne-Laure Chong
- Cancer Research Program, Research Institute of the McGill University Health Centre, Montreal, Canada
- Cancer Axis, Lady Davis Institute for Medical Research, Jewish General Hospital, Montreal, Canada
| | - Maria Apellaniz-Ruiz
- Cancer Axis, Lady Davis Institute for Medical Research, Jewish General Hospital, Montreal, Canada
- Department of Human Genetics, McGill University, Montreal, Canada
- Gerald Bronfman Department of Oncology, McGill University, Montreal, Quebec
| | - Naciba Benlimame
- Research Pathology Facility, Lady Davis Institute, Jewish General Hospital, Montreal, Canada
| | - Paula Marrano
- Department of Pediatric Laboratory Medicine, Hospital for Sick Children, Toronto
| | - Fadi Brimo
- Department of Pathology, McGill University Health Centre, Montreal, QC, Canada
| | - Somruetai Shuangshoti
- Institute of Pathology, Department of Medical Services, Ministry of Public Health, Bangkok, Thailand
| | - Shanop Shuangshoti
- Department of Pathology and Chulalongkorn GenePRO Center, Faculty of Medicine, Chulalongkorn University, Bangkok, Thailand
| | - William D Foulkes
- Cancer Research Program, Research Institute of the McGill University Health Centre, Montreal, Canada
- Cancer Axis, Lady Davis Institute for Medical Research, Jewish General Hospital, Montreal, Canada
- Department of Human Genetics, McGill University, Montreal, Canada
- Gerald Bronfman Department of Oncology, McGill University, Montreal, Quebec
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Silva LGDO, Lemos FFB, Luz MS, Rocha Pinheiro SL, Calmon MDS, Correa Santos GL, Rocha GR, de Melo FF. New avenues for the treatment of immunotherapy-resistant pancreatic cancer. World J Gastrointest Oncol 2024; 16:1134-1153. [PMID: 38660642 PMCID: PMC11037047 DOI: 10.4251/wjgo.v16.i4.1134] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/29/2023] [Revised: 01/26/2024] [Accepted: 03/04/2024] [Indexed: 04/10/2024] Open
Abstract
Pancreatic cancer (PC) is characterized by its extremely aggressive nature and ranks 14th in the number of new cancer cases worldwide. However, due to its complexity, it ranks 7th in the list of the most lethal cancers worldwide. The pathogenesis of PC involves several complex processes, including familial genetic factors associated with risk factors such as obesity, diabetes mellitus, chronic pancreatitis, and smoking. Mutations in genes such as KRAS, TP53, and SMAD4 are linked to the appearance of malignant cells that generate pancreatic lesions and, consequently, cancer. In this context, some therapies are used for PC, one of which is immunotherapy, which is extremely promising in various other types of cancer but has shown little response in the treatment of PC due to various resistance mechanisms that contribute to a drop in immunotherapy efficiency. It is therefore clear that the tumor microenvironment (TME) has a huge impact on the resistance process, since cellular and non-cellular elements create an immunosuppressive environment, characterized by a dense desmoplastic stroma with cancer-associated fibroblasts, pancreatic stellate cells, extracellular matrix, and immunosuppressive cells. Linked to this are genetic mutations in TP53 and immunosuppressive factors that act on T cells, resulting in a shortage of CD8+ T cells and limited expression of activation markers such as interferon-gamma. In this way, finding new strategies that make it possible to manipulate resistance mechanisms is necessary. Thus, techniques such as the use of TME modulators that block receptors and stromal molecules that generate resistance, the use of genetic manipulation in specific regions, such as microRNAs, the modulation of extrinsic and intrinsic factors associated with T cells, and, above all, therapeutic models that combine these modulation techniques constitute the promising future of PC therapy. Thus, this study aims to elucidate the main mechanisms of resistance to immunotherapy in PC and new ways of manipulating this process, resulting in a more efficient therapy for cancer patients and, consequently, a reduction in the lethality of this aggressive cancer.
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Affiliation(s)
| | - Fabian Fellipe Bueno Lemos
- Instituto Multidisciplinar em Saúde, Universidade Federal da Bahia, Vitória da Conquista 45029-094, Bahia, Brazil
| | - Marcel Silva Luz
- Instituto Multidisciplinar em Saúde, Universidade Federal da Bahia, Vitória da Conquista 45029-094, Bahia, Brazil
| | - Samuel Luca Rocha Pinheiro
- Instituto Multidisciplinar em Saúde, Universidade Federal da Bahia, Vitória da Conquista 45029-094, Bahia, Brazil
| | - Mariana dos Santos Calmon
- Instituto Multidisciplinar em Saúde, Universidade Federal da Bahia, Vitória da Conquista 45029-094, Bahia, Brazil
| | - Gabriel Lima Correa Santos
- Instituto Multidisciplinar em Saúde, Universidade Federal da Bahia, Vitória da Conquista 45029-094, Bahia, Brazil
| | - Gabriel Reis Rocha
- Instituto Multidisciplinar em Saúde, Universidade Federal da Bahia, Vitória da Conquista 45029-094, Bahia, Brazil
| | - Fabrício Freire de Melo
- Instituto Multidisciplinar em Saúde, Universidade Federal da Bahia, Vitória da Conquista 45029-094, Bahia, Brazil
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Jung HS, Lee M, Han Y, Thomas AS, Yun WG, Cho YJ, Kluger MD, Jang JY, Kwon W. Inadequacy of the eighth edition of the American Joint Committee on Cancer pancreatic cancer staging system for invasive carcinoma associated with premalignant lesions in the pancreas: an analysis using the National Cancer Database. HPB (Oxford) 2024; 26:400-409. [PMID: 38114399 DOI: 10.1016/j.hpb.2023.11.015] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/25/2023] [Revised: 09/09/2023] [Accepted: 11/28/2023] [Indexed: 12/21/2023]
Abstract
BACKGROUND Invasive carcinomas arising from premalignant lesions are currently staged by the same criteria as conventional pancreatic ductal adenocarcinoma. METHODS Clinicopathologic information and survival data were extracted through a thorough search of histology codes from National Cancer Database (2006-2016). A total of 723 patients with invasive intraductal papillary mucinous neoplasm and mucinous cystic neoplasm were analyzed. RESULTS The median age was 67 years, and 351 patients (48.5%) were male. There were 212 (29.3%), 232 (32.1%), 272 (37.6%), and 7 (1.0%) patients with T1, T2, T3, and T4 classification. Extrapancreatic extension (EPE) was present in 284 (39.3%). Age (HR = 1.504, 95% CI 1.196-1.891), R1 or R2 resection (HR = 1.585, 95% CI 1.175-2.140), and EPE (HR = 1.598, 95% CI 1.209-2.113) were independent prognostic factors for overall survival. Size criteria did not significantly affect survival. The median survival was 115.9 months for patients without EPE, compared to 34.2 months for those with EPE. EPE discriminated survival better than tumor size. DISCUSSION The T classification of the eighth edition AJCC staging system is not adequate for invasive carcinomas associated with premalignant lesions of the pancreas. They merit a separate, dedicated staging system that uses appropriate prognostic factors.
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Affiliation(s)
- Hye-Sol Jung
- Department of Surgery and Cancer Research Institute, Seoul National University College of Medicine, Seoul, South Korea
| | - Mirang Lee
- Department of Surgery and Cancer Research Institute, Seoul National University College of Medicine, Seoul, South Korea
| | - Youngmin Han
- Department of Surgery and Cancer Research Institute, Seoul National University College of Medicine, Seoul, South Korea
| | - Alexander S Thomas
- Division of GI/Endocrine Surgery, Department of Surgery, Columbia University Vagelos College of Physicians and Surgeons, New York, USA
| | - Won-Gun Yun
- Department of Surgery and Cancer Research Institute, Seoul National University College of Medicine, Seoul, South Korea
| | - Young J Cho
- Department of Surgery and Cancer Research Institute, Seoul National University College of Medicine, Seoul, South Korea
| | - Michael D Kluger
- Division of GI/Endocrine Surgery, Department of Surgery, Columbia University Vagelos College of Physicians and Surgeons, New York, USA
| | - Jin-Young Jang
- Department of Surgery and Cancer Research Institute, Seoul National University College of Medicine, Seoul, South Korea
| | - Wooil Kwon
- Department of Surgery and Cancer Research Institute, Seoul National University College of Medicine, Seoul, South Korea.
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11
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Yamashita Y, Shimokawa T, Ashida R, Hirooka Y, Iwashita T, Kato H, Kin T, Masamune A, Miwa H, Ohno E, Shiomi H, Sofuni A, Takenaka M, Kitano M. Protocol for a Multi-Center Confirmatory Trial to Evaluate the Differential Diagnostic Performance of Contrast-Enhanced Ultrasonography Using Perflubutane in Patients with a Pancreatic Mass: A Multicenter Prospective Study. Diagnostics (Basel) 2024; 14:130. [PMID: 38248007 PMCID: PMC10814130 DOI: 10.3390/diagnostics14020130] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/14/2023] [Revised: 12/09/2023] [Accepted: 12/11/2023] [Indexed: 01/23/2024] Open
Abstract
For pancreatic masses, an evaluation of their vascularity using contrast-enhanced ultrasonography can help improve their characterization. This study was designed to evaluate the utility and safety of contrast-enhanced transabdominal ultrasonography (CE-TUS) and endoscopic ultrasonography (CE-EUS) in the diagnosis of pancreatic masses including solid or cystic masses. This multi-center comparative open-label superiority study is designed to compare Plain (P)-TUS/EUS alone with P-TUS/P-EUS plus CE-TUS/CE-EUS. Three hundred and one patients with a total of 232 solid pancreatic masses and 69 cystic masses were prospectively enrolled. The primary endpoints are to compare the diagnostic accuracy between P-TUS/P-EUS alone and P-TUS/P-EUS plus CE-TUS/CE-EUS for both the TUS and EUS of solid pancreatic masses, and to compare the diagnostic accuracy between P-EUS alone and P-EUS plus CE-EUS in cystic pancreatic masses. The secondary endpoints are to compare the diagnostic sensitivity and specificity of P-TUS/P-EUS alone and P-TUS/P-EUS plus CE-TUS/CE-EUS for pancreatic solid/cystic masses, and the accuracy of P-TUS alone and P-TUS plus CE-TUS for pancreatic cystic masses. Other secondary endpoints included comparing the diagnostic sensitivity, specificity, and accuracy of CE-TUS, CE-EUS and CE-computed tomography (CT) for solid/cystic pancreatic masses. The safety, degree of effective enhancement, and diagnostic confidence obtained with CE-TUS/CE-EUS will also be assessed.
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Affiliation(s)
- Yasunobu Yamashita
- Second Department of Internal Medicine, Wakayama Medical University, 811-1 Kimiidera, Wakayama 641-0012, Japan
| | - Toshio Shimokawa
- Clinical Study Support Center, Wakayama Medical University Hospital, Wakayama 641-0012, Japan
| | - Reiko Ashida
- Second Department of Internal Medicine, Wakayama Medical University, 811-1 Kimiidera, Wakayama 641-0012, Japan
| | - Yoshiki Hirooka
- Department of Gastroenterology and Gastroenterological Oncology, Fujita Health University School of Medicine, Toyoake 470-1192, Japan
| | - Takuji Iwashita
- First Department of Internal Medicine, Gifu University Hospital, Gifu 501-1194, Japan
| | - Hironari Kato
- Department of Gastroenterology and Hepatology, Okayama University Graduate School of Medicine, Okayama 700-8558, Japan
| | - Toshifumi Kin
- Center for Gastroenterology, Teine Keijinkai Hospital, Sapporo 006-8555, Japan
| | - Atsushi Masamune
- Division of Gastroenterology, Tohoku University Graduate School of Medicine, Toyoake 980-8574, Japan
| | - Haruo Miwa
- Gastroenterological Center, Yokohama City University Medical Center, Yokohama 232-0024, Japan
| | - Eizaburo Ohno
- Department of Gastroenterology and Hepatology, University Graduate School of Medicine, Nagoya 466-8550, Japan
| | - Hideyuki Shiomi
- Division of Gastroenterology and Hepatobiliary and Pancreatic Diseases, Department of Internal Medicine, Hyogo Medical University, Nishinomiya 663-8501, Japan
| | - Atsushi Sofuni
- Department of Gastroenterology and Hepatology, Tokyo Medical University, Tokyo 160-0023, Japan
| | - Mamoru Takenaka
- Department of Gastroenterology and Hepatology, Kindai University Faculty of Medicine, Osaka 589-8511, Japan
| | - Masayuki Kitano
- Second Department of Internal Medicine, Wakayama Medical University, 811-1 Kimiidera, Wakayama 641-0012, Japan
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12
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Mattiolo P, Wang H, Basturk O, Brosens LAA, Hong SM, Adsay V, Scarpa A, Luchini C. Comprehensive characterisation of acinar cystic transformation of the pancreas: a systematic review. J Clin Pathol 2023; 76:740-746. [PMID: 37643836 DOI: 10.1136/jcp-2023-209103] [Citation(s) in RCA: 2] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/01/2023] [Accepted: 08/16/2023] [Indexed: 08/31/2023]
Abstract
AIMS Acinar cystic transformation (ACT) of the pancreas is a rare pancreatic cystic lesion. Owing to its rarity, comprehensive histomolecular characterisation of this entity is still lacking. We aim to perform a systematic review on this controversial entity. METHODS We searched PubMed, SCOPUS and Embase through May 2023 to identify all studies on ACTs. Clinicopathological, immunohistochemical (IHC) and molecular data have been extracted and analysed. RESULTS Overall, there were 121 cases of ACTs in the literature. ACT had a female predominance (65.3% of patients), and a mean size of 4.8 cm. ACT was more often unifocal (71.9%) and multiloculate (61.2%). Histologically, the cysts were lined by an acinar epithelium, sometimes harbouring ductal-like areas (18.2%). In five cases (4.1%), an intralesional pancreatic intraepithelial neoplasia (PanIN) was reported. Preoperative diagnosis is challenging. After surgical resection, all patients were alive and disease free during follow-up except one patient who developed a second ACT after resection. By IHC, all lesions were positive for acinar markers; cytokeratin 7 and 8/18/19 were usually positive, and Ki-67 was invariably ≤3%. At the molecular level, three cases demonstrated genetic alterations: one showed multiple chromosomal gains, and other two harboured somatic mutations of KRAS and SMO genes (one mutation per case). CONCLUSIONS Globally considered, our findings demonstrated that ACT is a benign entity, without the need of surgical resection with the exception of symptomatic lesions. The rare occurrence of intracystic PanINs and driver mutations suggest considering follow-up if a preoperative diagnosis of ACT can be made.
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Affiliation(s)
- Paola Mattiolo
- Department of Diagnostics and Public Health, Section of Pathology, University and Hospital Trust of Verona, Verona, Italy
| | - Huamin Wang
- Department of Anatomical Pathology, University of Texas MD Anderson Cancer Center, Houston, Texas, USA
| | - Olca Basturk
- Department of Pathology, Memorial Sloan Kettering Cancer Center, New York, New York, USA
| | | | - Seung-Mo Hong
- Department of Pathology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea (the Republic of)
| | - Volkan Adsay
- Department of Pathology, Koç University Hospital and Koç University Research Center for Translational Medicine (KUTTAM), Istanbul, Turkey
| | - Aldo Scarpa
- Department of Diagnostics and Public Health, Section of Pathology, and ARC-Net Research Center, University and Hospital Trust of Verona, Verona, Italy
| | - Claudio Luchini
- Department of Diagnostics and Public Health, Section of Pathology, and ARC-Net Research Center, University and Hospital Trust of Verona, Verona, Italy
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13
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Pollini T, Marchegiani G, Facciorusso A, Balduzzi A, Biancotto M, Bassi C, Maker AV, Salvia R. It is not necessary to resect all mucinous cystic neoplasms of the pancreas: current guidelines do not reflect the actual risk of malignancy. HPB (Oxford) 2023; 25:747-757. [PMID: 37003852 DOI: 10.1016/j.hpb.2023.03.001] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/29/2022] [Revised: 02/06/2023] [Accepted: 03/03/2023] [Indexed: 04/03/2023]
Abstract
BACKGROUND Mucinous Cystic Neoplasms (MCN) of the pancreas are premalignant cysts for which current guidelines support pancreatic resection. The primary aim of this systematic review and meta-analysis is to define the pooled rate of malignancy for MCN. METHODS A systematic review of eligible studies published between 2000 and 2021 was performed on PubMed and Embase. Primary outcome was rate of malignancy. Data regarding high-risk features, including cyst size and mural nodules, were collected and analyzed. RESULTS A total of 40 studies and 3292 patients with resected MCN were included in the final analysis. The pooled rate of malignancy was 16.1% (95%CI 13.1-19.0). The rate of malignant MCN in studies published before 2012 was significantly higher than that of studies published after recent guidelines were published (21.0% vs 14.9%, p < 0.001). Malignant MCN were larger than benign (mean difference 25.9 mm 95%CI 14.50-37.43, p < 0.001) with a direct correlation between size and presence of malignant MCN (R2 = 0.28, p = 0.020). A SROC identified a threshold of 65 mm to be associated with the diagnosis of malignant MCN. Presence of mural nodules was associated with the diagnosis of a malignant MCN (OR = 4.34, 95%CI 3.00-6.29, p < 0.001). CONCLUSION Whereas guidelines recommend resection of all MCN, the rate of malignancy in resected MCN is 16%, implying that surveillance has a role in most cases, and that surgical selection criteria are warranted. Size and presence of mural nodules are significantly associated with an increased risk of malignant degeneration, small MCN and without mural nodules can be considered for surveillance.
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Affiliation(s)
- Tommaso Pollini
- Division of Surgical Oncology, Department of Surgery, University of California San Francisco, San Francisco, USA; The Pancreas Institute, Department of General and Pancreatic Surgery, University of Verona, Verona, Italy
| | - Giovanni Marchegiani
- The Pancreas Institute, Department of General and Pancreatic Surgery, University of Verona, Verona, Italy
| | - Antonio Facciorusso
- Gastroenterology Unit, Department of Surgical and Medical Sciences, University of Foggia, Foggia, Italy
| | - Alberto Balduzzi
- The Pancreas Institute, Department of General and Pancreatic Surgery, University of Verona, Verona, Italy
| | - Marco Biancotto
- The Pancreas Institute, Department of General and Pancreatic Surgery, University of Verona, Verona, Italy
| | - Claudio Bassi
- The Pancreas Institute, Department of General and Pancreatic Surgery, University of Verona, Verona, Italy
| | - Ajay V Maker
- Division of Surgical Oncology, Department of Surgery, University of California San Francisco, San Francisco, USA
| | - Roberto Salvia
- The Pancreas Institute, Department of General and Pancreatic Surgery, University of Verona, Verona, Italy.
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14
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Megibow AJ. Pancreatic Cysts: Radiology. Gastrointest Endosc Clin N Am 2023; 33:519-531. [PMID: 37245933 DOI: 10.1016/j.giec.2023.03.008] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 05/30/2023]
Abstract
This article reviews the types of pancreatic cysts encountered in Radiologic practice. It summarizes the malignancy risk of each of the following: serous cystadenoma, mucinous cystic tumor, intraductal papillary mucinous neoplasm main duct and side branch, and some miscellaneous cysts such as neuroendocrine tumor and solid pseudopapillary epithelial neoplasm. Specific reporting recommendations are given. The choice between radiology follow-up versus endoscopic analysis is discussed.
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Affiliation(s)
- Alec J Megibow
- Department of Radiology, NYU-Langone Health, 550 1st Avenue, Room HCC 232, New York, NY 10016, USA.
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15
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Xia Q, Li F, Min R, Sun S, Han YX, Feng ZZ, Li N. Malignancy risk factors and prognostic variables of pancreatic mucinous cystic neoplasms in Chinese patients. World J Gastroenterol 2023; 29:3119-3132. [PMID: 37346158 PMCID: PMC10280801 DOI: 10.3748/wjg.v29.i20.3119] [Citation(s) in RCA: 2] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/03/2023] [Revised: 04/21/2023] [Accepted: 05/04/2023] [Indexed: 05/26/2023] Open
Abstract
BACKGROUND Pancreatic mucinous cystic neoplasms (MCNs) represent one of the precursor lesions of pancreatic ductal adenocarcinoma, and their detection has been facilitated by advances in preoperative imaging. Due primarily to the rarity of MCNs, however, there is limited knowledge regarding the prognostic variables and high-risk factors for malignant transformation. A more comprehensive and nuanced approach is necessary to fill this gap and provide a basis for improved treatment decisions and patient outcomes.
AIM To investigate the high-risk factors associated with malignant MCNs and to explore the prognostic factors of MCN with associated invasive carcinoma (MCN-AIC).
METHODS All cases of resected MCNs from a single high-volume institution between January 2012 and January 2022 were retrospectively reviewed. Only cases with ovarian-type stroma verified by progesterone receptor staining were included. Preoperative features, histological findings and postoperative course were documented. Multivariate logistic regression was employed to investigate variables related to malignancy. Survival analysis was performed using the Kaplan-Meier curve, and the prognostic factors were assessed to evaluate the postoperative course of patients with MCN-AIC.
RESULTS Among the 48 patients, 36 had benign MCNs, and 12 had malignant MCNs (1 high-grade atypical hyperplasia and 11 MCN-AIC). Age, tumour size, presence of solid components or mural nodules and pancreatic duct dilatation were identified as independent risk factors associated with malignancy. The follow-up period ranged from 12 mo to 120 mo, with a median overall survival of 58.2 mo. Only three patients with MCN-AIC died, and the 5-year survival rate was 70.1%. All 11 cases of MCN-AIC were stage I, and extracapsular invasion was identified as a prognostic factor for poorer outcomes.
CONCLUSION The risk factors independently associated with malignant transformation of MCNs included age, tumour size, presence of solid components or mural nodules, and pancreatic duct dilatation. Our study also revealed that encapsulated invasion was a favourable prognostic factor in MCN-AIC patients.
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MESH Headings
- Humans
- Carcinoma, Pancreatic Ductal/epidemiology
- Carcinoma, Pancreatic Ductal/pathology
- Carcinoma, Pancreatic Ductal/surgery
- East Asian People
- Neoplasms, Cystic, Mucinous, and Serous/epidemiology
- Neoplasms, Cystic, Mucinous, and Serous/pathology
- Neoplasms, Cystic, Mucinous, and Serous/surgery
- Pancreatic Neoplasms/epidemiology
- Pancreatic Neoplasms/pathology
- Pancreatic Neoplasms/surgery
- Prognosis
- Receptors, Progesterone
- Retrospective Studies
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Affiliation(s)
- Qing Xia
- Department of Pathology, The First Affiliated Hospital of Bengbu Medical College, Bengbu Medical College, Bengbu 233004, Anhui Province, China
| | - Fan Li
- Department of Pathology, Bengbu Medical College, Bengbu 233030, Anhui Province, China
| | - Rui Min
- Department of Pathology, The First Affiliated Hospital of Bengbu Medical College, Bengbu Medical College, Bengbu 233004, Anhui Province, China
| | - Shuai Sun
- Department of Pathology, Bengbu Medical College, Bengbu 233030, Anhui Province, China
| | - Yue-Xin Han
- School of Clinical Medicine, Bengbu Medical College, Bengbu 233030, Anhui Province, China
| | - Zhen-Zhong Feng
- Department of Pathology, The Second Affiliated Hospital of Anhui Medical University, Hefei 230601, Anhui Province, China
| | - Nan Li
- Department of Pathology, The First Affiliated Hospital of Bengbu Medical College, Bengbu Medical College, Bengbu 233004, Anhui Province, China
- Department of Pathology, The Second Affiliated Hospital of Anhui Medical University, Hefei 230601, Anhui Province, China
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16
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Hozaka Y, Ohtsuka T, Nakamura M, Hijioka S, Unno M, Shimizu Y, Tanabe M, Nagakawa Y, Hatano E, Kawai M, Sano T, Koshita S, Katanuma A, Kita E, Hanada K, Nakai Y, Aoki T, Serikawa M, Okamoto K, Takeyama Y. Feasibility of Surveillance for Mucinous Cystic Neoplasm of the Pancreas: A Multi-Institutional Retrospective Study of 328 Patients by the Japanese Pancreatic Society. Pancreas 2023; 52:e288-e292. [PMID: 37922344 DOI: 10.1097/mpa.0000000000002254] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/05/2023]
Abstract
OBJECTIVE We aimed to elucidate the feasibility of surveillance of patients with mucinous cystic neoplasm (MCN). METHODS We performed a retrospective, multi-institutional study of 328 patients who underwent surgery for MCN at 18 Japanese institutions. Patients with MCN were divided into an immediate surgery group and a surveillance group, which underwent surgery after surveillance. RESULTS The median surveillance period until surgery in the surveillance group was 27 months (range, 7-165 months). Compared with the immediate surgery group, the surveillance group showed smaller tumor diameter (46 vs 50 mm, P = 0.01), more frequent laparoscopic approach (58% vs 37%, P < 0.01), and less frequent malignancy (7% vs 15%, P = 0.03). The new appearance of mural nodules and elevation of serum tumor markers were associated with malignancy in the surveillance group. Two patients in the surveillance group experienced postoperative recurrence, although there was no significant difference in recurrence or disease-free survival between the two groups. In the surveillance group, the 1-, 5-, and 10-year cumulative incidence rates of malignant MCN were 0.8%, 5.6%, and 36.5%, respectively. CONCLUSION As the risk of progression to malignant MCNs increases over the long term, MCNs should be resected rather than subjected to unnecessary surveillance.
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Affiliation(s)
- Yuto Hozaka
- From the Department of Digestive Surgery, Breast and Thyroid Surgery, Graduate School of Medical and Dental Sciences, Kagoshima University, Kagoshima, Japan
| | - Takao Ohtsuka
- From the Department of Digestive Surgery, Breast and Thyroid Surgery, Graduate School of Medical and Dental Sciences, Kagoshima University, Kagoshima, Japan
| | - Masafumi Nakamura
- Department of Surgery and Oncology, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan
| | - Susumu Hijioka
- Department of Hepatobiliary and Pancreatic Oncology, National Cancer Center Hospital, Tokyo, Japan
| | - Michiaki Unno
- Department of Surgery, Tohoku University Graduate School of Medicine, Sendai, Japan
| | - Yasuhiro Shimizu
- Department of Gastroenterological Surgery, Aichi Cancer Center Hospital, Aichi, Japan
| | - Minoru Tanabe
- Department of Hepatobiliary and Pancreatic Surgery, Graduate School of Medicine, Tokyo Medical and Dental University, Tokyo, Japan
| | - Yuichi Nagakawa
- Department of Gastrointestinal and Pediatric Surgery, Tokyo Medical University, Tokyo, Japan
| | - Etsuro Hatano
- Division of Hepato-Biliary-Pancreatic Surgery and Transplantation, Department of Surgery, Graduate School of Medicine, Kyoto University, Kyoto, Japan
| | - Manabu Kawai
- Second Department of Surgery, Wakayama Medical University, Wakayama, Japan
| | - Tsuyoshi Sano
- Division of Gastroenterological Surgery, Department of Surgery, Aichi Medical University, Aichi, Japan
| | - Shinsuke Koshita
- Department of Gastroenterology, Sendai City Medical Center, Miyagi, Japan
| | - Akio Katanuma
- Center for Gastroenterology, Teine Keijinkai Hospital, Hokkaido, Japan
| | - Emiri Kita
- Department of Gastroenterology, Chiba Cancer Center, Chiba, Japan
| | - Keiji Hanada
- Department of Gastroenterology, Onomichi General Hospital, Onomichi, Japan
| | | | - Takeshi Aoki
- Department of Gastroenterological and General Surgery, School of Medicine, Showa University, Tokyo, Japan
| | - Masahiro Serikawa
- Department of Gastroenterology, Graduate School of Biomedical and Health Sciences, Hiroshima University, Hiroshima, Japan
| | - Kojun Okamoto
- Department of Gastroenterological Surgery, Saitama Medical University International Medical Center, Saitama, Japan
| | - Yoshifumi Takeyama
- Department of Surgery, Kinki University Faculty of Medicine, Osaka, Japan
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17
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Alomair AA, Almohaimeed RA, Alsaud JS, Alotaibi DF, Alharbi OT. Mucinous Cystic Neoplasm of the Pancreas in a Pregnant Woman: A Case Report and Review of the Literature. Cureus 2023; 15:e37787. [PMID: 37214006 PMCID: PMC10195075 DOI: 10.7759/cureus.37787] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 04/15/2023] [Indexed: 05/23/2023] Open
Abstract
Mucinous cystic neoplasms of the pancreas are rare tumors that represent 10% of cystic pancreatic tumors. They are potentially sex hormone-sensitive. However, mucinous cystic neoplasms occurring during pregnancy are relatively uncommon. A 33-year-old woman in her ninth week of gestation was referred to us due to abdominal pain for two months. Magnetic resonance imaging revealed a well-defined unilocular cystic lesion at the tail of the pancreas, measuring 7x6.4 cm. The patient underwent tumor resection with distal pancreatectomy and splenectomy during the second trimester to prevent the potential risk of rupture of the neoplasm, rapid growth, and/or intrauterine growth restriction. Histopathological examination revealed a mucinous cystadenoma with no atypia or malignancy. The patient completely recovered from the surgery and had a healthy full-term baby. This case shows the benefit of performing the surgery during the second trimester compared to the potential risk of delaying the surgery.
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18
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Dhamor D, Irrinki S, Naik A, Kurdia KC, Rastogi P, Gupta P, Kapoor VK. Pregnancy-associated mucinous cystic neoplasms of the pancreas - A systematic review. Am J Surg 2023; 225:630-638. [PMID: 36424200 DOI: 10.1016/j.amjsurg.2022.11.002] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/29/2022] [Accepted: 11/02/2022] [Indexed: 11/18/2022]
Abstract
INTRODUCTION Mucinous cystic neoplasms (MCN) are mucin-producing epithelial cell tumors of pancreas. They consist of an ovarian-type stroma expressing estrogen and progesterone receptors. Pregnancy-associated MCNs are presumed to be larger in size and more aggressive without any concrete evidence. OBJECTIVE and Data Sources: Systematic review of published literature using PubMed and Google Scholar databases. Original articles including case reports and series published between 1970&2021 were included wherein MCN was diagnosed during pregnancy/within one-year post-partum. Thirty-three publications having 36 cases, adding one of our own patient were analyzed in this review. RESULT Median age at presentation was 32 years. Only three (9%) patients were asymptomatic. Mean size of MCN was 135 mm. Ten patients (27%) reported an increase in size during pregnancy. Most tumors involved body and tail of pancreas (60%). Distal pancreatectomy with splenectomy was the most common resection performed (57%). No foetal mortality was reported to date. CONCLUSION Pregnancy may cause a rapid increase in size of MCN. Decision-making is more complex and needs a fine balance between optimal oncological and obstetric outcomes.
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Affiliation(s)
- Devesh Dhamor
- Department of General Surgery, Post Graduate Institute of Medical Education and Research, Chandigarh, India
| | - Santhosh Irrinki
- Department of General Surgery, Post Graduate Institute of Medical Education and Research, Chandigarh, India
| | - Anil Naik
- Department of General Surgery, Post Graduate Institute of Medical Education and Research, Chandigarh, India
| | - Kailash Chand Kurdia
- Department of General Surgery, Post Graduate Institute of Medical Education and Research, Chandigarh, India.
| | - Pulkit Rastogi
- Department of Haematology, Post Graduate Institute of Medical Education and Research, Chandigarh, India
| | - Pankaj Gupta
- Department of Radiodiagnosis and Imaging, Post Graduate Institute of Medical Education and Research, Chandigarh, India
| | - Vinay K Kapoor
- Department of Hepato-pancreato-biliary (HPB) Surgery, Mahatma Gandhi Medical College and Hospital (MGMCH), Jaipur, India
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19
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Horiuchi A, Akehi S, Abe Y, Ichikawa N, Kawaharada S, Kitazawa S, Kitazawa R. Mucinous cystadenoma of the pancreas associated with pancreatic pseudocyst. J Surg Case Rep 2023; 2023:rjad026. [PMID: 36755935 PMCID: PMC9902206 DOI: 10.1093/jscr/rjad026] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/23/2022] [Accepted: 01/14/2023] [Indexed: 02/08/2023] Open
Abstract
Mucinous cystadenoma of the pancreas is considered as a premalignant lesion, and resection is recommended. The majority of pancreatic cystic lesions are pancreatic pseudocysts, so differentiation between mucinous cystadenoma and pseudocyst is frequently required. We report a rare case of mucinous cystadenoma of the pancreas coexisting with pseudocyst. A 43-year-old woman presented with abdominal pain. Imaging examinations showed a large cystic lesion in the tail of the pancreas, and distal pancreatectomy and splenectomy were performed. Pathological examination revealed that the majority of the cystic wall comprised thick collagen fibrous connective tissue, while part of the cystic wall represented a single layer of columnar, mucin-producing epithelium without atypia. Those findings suggested mucinous cystadenoma with an inflammatory pseudocyst. The mixture of mucinous cystadenoma and pseudocyst within the same cystic lesion appears to be very rare. Complete resection of the cystic lesion seems to allow an excellent prognosis.
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Affiliation(s)
- Atsushi Horiuchi
- Correspondence address. 3-1-1 Hongou, Niihama City, Ehime 792-0042, Japan. Tel: +81-897-43-6161; Fax: +81-897-41-2900; E-mail:
| | - Shun Akehi
- Department of General Surgery, Ehime Prefectural Niihama Hospital, Niihama City, Ehime, Japan
| | - Yousuke Abe
- Department of General Surgery, Ehime Prefectural Niihama Hospital, Niihama City, Ehime, Japan
| | - Nanako Ichikawa
- Department of General Surgery, Ehime Prefectural Niihama Hospital, Niihama City, Ehime, Japan
| | - Sakura Kawaharada
- Department of General Surgery, Ehime Prefectural Niihama Hospital, Niihama City, Ehime, Japan
| | - Sohei Kitazawa
- Department of Molecular Pathology, Ehime University Graduate School of Medicine, Toon City, Ehime, Japan
| | - Riko Kitazawa
- Department of Molecular Pathology, Ehime University Graduate School of Medicine, Toon City, Ehime, Japan
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20
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Singh RR, Gopakumar H, Sharma NR. Diagnosis and Management of Pancreatic Cysts: A Comprehensive Review of the Literature. Diagnostics (Basel) 2023; 13:diagnostics13030550. [PMID: 36766654 PMCID: PMC9914101 DOI: 10.3390/diagnostics13030550] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/23/2022] [Revised: 01/20/2023] [Accepted: 01/23/2023] [Indexed: 02/05/2023] Open
Abstract
The prevalence of pancreatic cysts has been rising due to the widespread use of cross-sectional imaging (CT scan and MRI) of the abdomen. While most pancreatic cysts are benign and do not require treatment or surveillance, a significant minority are premalignant and rarely malignant. The risk stratification of these lesions is not straightforward, and individual risk assessment, cyst size, distribution, and alarming morphologic features (when present) can guide the next steps in management. Neoplastic pancreatic cysts are mucinous or non-mucinous. Endoscopic ultrasound with fine-needle aspiration is often required to classify pancreatic cysts into mucinous and non-mucinous cysts and to assess the malignant potential. Advances in endoscopic techniques (confocal laser endomicroscopy, microforceps biopsy) can provide a definitive diagnosis of pancreatic cysts in some cases; however, the use of these techniques involves a higher risk of adverse events.
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Affiliation(s)
- Ritu R. Singh
- Parkview Cancer Institute (PCI), Parkview Regional Medical Center, Fort Wayne, IN 46845, USA
- Department of Medicine, Indiana University School of Medicine, Fort Wayne, IN 46805, USA
| | - Harishankar Gopakumar
- Division of Gastroenterology and Hepatology, University of Illinois College of Medicine, Peoria, IL 61656, USA
| | - Neil R. Sharma
- Parkview Cancer Institute (PCI), Parkview Regional Medical Center, Fort Wayne, IN 46845, USA
- Department of Medicine, Indiana University School of Medicine, Fort Wayne, IN 46805, USA
- Correspondence:
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21
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Oyama K, Iwagami Y, Kobayashi S, Sasaki K, Yamada D, Tomimaru Y, Akita H, Noda T, Asaoka T, Nakahira S, Gotoh K, Takahashi H, Tanemura M, Doki Y, Eguchi H. A Ruptured Mucinous Cystadenocarcinoma of the Pancreas Extensively Evaluated Before and After the Rupture: A Case Report. Pancreas 2023; 52:e163-e167. [PMID: 37523608 DOI: 10.1097/mpa.0000000000002198] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 08/02/2023]
Abstract
ABSTRACT Pancreatic mucinous cystic neoplasm (MCN) rarely ruptures because of their surrounding fibrotic capsules and has never been reported with detailed information regarding prerupture and postrupture states. We report a case of MCN rupture where performed emergency surgery was performed while waiting for elective surgery. A 54-year-old woman was referred to our department for a pancreatic cystic tumor with slight abdominal pain. A cystic tumor with a nodular lesion was found, with a contrast effect measuring 78 mm in diameter. On day 21, the patient visited our hospital complaining of increased abdominal pain, but few signs of peritonitis were observed. Tests conducted revealed moderate ascites, marginal shrinkage of the cyst diameter, and a slight elevation of inflammatory markers. We suspected an MCN rupture and immediately performed distal pancreatectomy. Brown turbid ascites and rupture of the anterior wall of the cyst were observed. In the ascites, amylase levels were not elevated, and bacterial cultures were negative. The histopathological diagnosis was noninvasive mucinous cystadenocarcinoma. At 9 months after surgery, she started chemotherapy because of a recurrence of the peritoneal dissemination. This case provided valuable insight into the rupture of MCNs using thorough imaging techniques, laboratory, and physical findings before and after rupturing.
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Affiliation(s)
- Keisuke Oyama
- From the Department of Gastroenterological Surgery, Graduate School of Medicine, Osaka University
| | - Yoshifumi Iwagami
- From the Department of Gastroenterological Surgery, Graduate School of Medicine, Osaka University
| | - Shogo Kobayashi
- From the Department of Gastroenterological Surgery, Graduate School of Medicine, Osaka University
| | - Kazuki Sasaki
- From the Department of Gastroenterological Surgery, Graduate School of Medicine, Osaka University
| | - Daisaku Yamada
- From the Department of Gastroenterological Surgery, Graduate School of Medicine, Osaka University
| | - Yoshito Tomimaru
- From the Department of Gastroenterological Surgery, Graduate School of Medicine, Osaka University
| | - Hirofumi Akita
- Department of Gastroenterological Surgery, Osaka International Cancer Institute
| | - Takehiro Noda
- From the Department of Gastroenterological Surgery, Graduate School of Medicine, Osaka University
| | | | - Shin Nakahira
- From the Department of Gastroenterological Surgery, Graduate School of Medicine, Osaka University
| | - Kunihito Gotoh
- Department of Gastroenterological Surgery, National Hospital Organization Osaka National Hospital
| | - Hidenori Takahashi
- From the Department of Gastroenterological Surgery, Graduate School of Medicine, Osaka University
| | | | - Yuichiro Doki
- From the Department of Gastroenterological Surgery, Graduate School of Medicine, Osaka University
| | - Hidetoshi Eguchi
- From the Department of Gastroenterological Surgery, Graduate School of Medicine, Osaka University
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22
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Kriger AG, Panteleev VI, Dzhigkaeva MM, Budzinskiy SA. [Pancreatic mucinous cystadenoma with atypical clinical presentation]. Khirurgiia (Mosk) 2023:118-122. [PMID: 38088849 DOI: 10.17116/hirurgia2023121118] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/18/2023]
Abstract
We present a 33-year-old patient with atypical clinical course of pancreatic mucinous cystadenoma. The tumor had connection with pancreatic ductal system and led to bleeding into cystic cavity. This contributed to incorrect preoperative diagnosis of post-necrotic cyst. The final diagnosis of mucinous cystadenoma was established after histological examination. Distal pancreatectomy excluded incorrect treatment.
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Affiliation(s)
- A G Kriger
- Russian Research Radiology Center, Moscow, Russia
- Russian Medical Academy of Postgraduate Education, Moscow, Russia
| | | | | | - S A Budzinskiy
- Moscow City Clinical Hospital No. 31, Moscow, Russia
- Pirogov Russian National Research Medical University, Moscow, Russia
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23
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Tang H, Shen Z, Lu B. Huge mucinous cystic neoplasms with adhesion to the left colon: A case report and literature review. Open Med (Wars) 2022; 17:2130-2137. [PMID: 36660019 PMCID: PMC9816456 DOI: 10.1515/med-2022-0612] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/10/2021] [Revised: 10/25/2022] [Accepted: 11/02/2022] [Indexed: 01/04/2023] Open
Abstract
Mucinous cystic neoplasms (MCN) are rare premalignant neoplasms of the pancreas typically found as single lesions in the pancreatic body and tail of women in the fifth and sixth decade of life, constituting 2-5% of pancreatic neoplasms. We present a 50-year-old female patient with a large tail mass of the pancreatic body (size of 15 cm × 12 cm) with elevated tumor indicators. Computed tomography and magnetic resonance imaging revealed a large cystic lesion with septa and wall nodules. During the operation, the tumor capsule wall adhered to the left half colon and mesentery and could not be detached. Splenectomy and left hemicolectomy were performed. The postoperative pathological examination of the specimens confirmed a premalignant pancreatic mucous cystic tumor with moderate heterocytosis. The preoperative diagnosis of pancreatic MCN and MCN with invasive carcinoma is discussed, considering the characteristics of this case. Age, tumor size, texture, tumor marker elevation, and cystic wall condition are important characteristics of malignant MCN. Nevertheless, it is still very difficult to determine accurately whether an MCN is malignant or not before an actual pathological examination of the resected specimen.
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Affiliation(s)
- Haijun Tang
- Hepatopancreatobiliary Surgery Department, Shaoxing People’s Hospital, Shaoxing, Zhejiang, 312000, China
| | - Zhihong Shen
- Hepatopancreatobiliary Surgery Department, Shaoxing People’s Hospital, Shaoxing, Zhejiang, 312000, China
| | - Baochun Lu
- Hepatopancreatobiliary Surgery Department, Shaoxing People’s Hospital, Shaoxing, Zhejiang, 312000, China
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24
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Chen W, Ahmed N, Krishna SG. Pancreatic Cystic Lesions: A Focused Review on Cyst Clinicopathological Features and Advanced Diagnostics. Diagnostics (Basel) 2022; 13:65. [PMID: 36611356 PMCID: PMC9818257 DOI: 10.3390/diagnostics13010065] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.7] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/16/2022] [Revised: 12/13/2022] [Accepted: 12/19/2022] [Indexed: 12/28/2022] Open
Abstract
Macroscopic, endomicroscopic, and histologic findings and correlation are an integral part of the diagnostic evaluation of pancreatic cystic lesions (PCLs), as complementing morphologic features seen by different specialties are combined to contribute to a final diagnosis. However, malignancy risk stratification of PCLs with worrisome features can still be challenging even after endoscopic ultrasound guided-fine needle aspiration (EUS-FNA) with cytological evaluation. This review aims to summarize cyst clinicopathological features from the pathologists' perspective, coupled with knowledge from advanced diagnostics-confocal laser endomicroscopy and cyst fluid molecular analysis, to demonstrate the state-of-art risk stratification of PCLs. This review includes illustrative photos of surgical specimens, endomicroscopic and histologic images, and a summary of cyst fluid molecular markers.
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Affiliation(s)
- Wei Chen
- Department of Pathology, The Ohio State University Wexner Medical Center, Columbus, OH 43210, USA
| | - Nehaal Ahmed
- School of Medicine, The Ohio State University, Columbus, OH 43210, USA
| | - Somashekar G. Krishna
- Division of Gastroenterology, Department of Internal Medicine, The Ohio State University Wexner Medical Center, Columbus, OH 43210, USA
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25
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Bell PD, Singhi AD. Integrating Molecular Analysis into the Pathologic Evaluation of Pancreatic Cysts. Surg Pathol Clin 2022; 15:455-468. [PMID: 36049828 DOI: 10.1016/j.path.2022.05.009] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/15/2022]
Abstract
The development of cross-sectional imaging techniques has enhanced the detection of pancreatic cystic lesions (PCLs). PCLs are found in approximately 2% of the general population, often as incidentally detected lesions on computed tomography or MRI during the evaluation of other medical conditions. Broadly, PCLs are classified as mucinous or nonmucinous. Mucinous PCLs include mucinous cystic neoplasms and intraductal papillary mucinous neoplasms. Nonmucinous PCLs include pseudocysts, serous cystadenomas, solid pseudopapillary neoplasms, and cystic pancreatic neuroendocrine tumors, as well as cystic acinar cell carcinoma, cystic degeneration of pancreatic ductal adenocarcinoma, lymphoepithelial cyst, and others.
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Affiliation(s)
- Phoenix D Bell
- Department of Pathology, University of Pittsburgh Medical Center, 200 Lothrop St. Pittbsurgh, PA 15213, USA.
| | - Aatur D Singhi
- Department of Pathology, University of Pittsburgh Medical Center, 200 Lothrop St. Pittbsurgh, PA 15213, USA
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26
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Kim GH, Choi K, Paik N, Lee KT, Lee JK, Lee KH, Han IW, Kang SH, Heo JS, Park JK. Diagnostic Concordance and Preoperative Risk Factors for Malignancy in Pancreatic Mucinous Cystic Neoplasms. Gut Liver 2022; 16:637-644. [PMID: 34933278 PMCID: PMC9289824 DOI: 10.5009/gnl210231] [Citation(s) in RCA: 4] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/23/2021] [Revised: 08/24/2021] [Accepted: 09/03/2021] [Indexed: 11/04/2022] Open
Abstract
BACKGROUND/AIMS As pancreatic mucinous cystic neoplasms (MCNs) are considered premalignant lesions, the current guidelines recommend their surgical resection. We aimed to investigate the concordance between preoperative and postoperative diagnoses and evaluate preoperative clinical parameters that could predict the malignant potential of MCNs. METHODS Patients who underwent surgical resection at Samsung Medical Center for pancreatic cystic lesions and whose pathology was confirmed to be MCN, between July 2000 and December 2017, were retrospectively analyzed. RESULTS Among a total of 132 patients 99 (75%) were diagnosed with MCN preoperatively. The most discordant preoperative diagnosis was an indeterminate pancreatic cyst. The proportion of male patients was higher (24.2% vs 7.1%, p=0.05) in the diagnosis-discordance group and the presence of worrisome features in radiologic imaging studies, such as wall thickening/enhancement (12.1% vs 37.4%, p=0.02) or solid component/mural nodule (3.0% vs 27.3%, p=0.02), was lower in the diagnosis-discordance group. The presence of symptoms (57.7% vs 34.9%, p=0.02), tumor size greater than 4 cm (80.8% vs 55.7%, p=0.04), and radiologic presence of a solid component/mural nodule (42.3% vs 16.0%, p=0.01) or duct dilatation (19.2% vs 6.6%, p=0.01) were significantly associated with malignant MCNs. CONCLUSIONS In our study, the overall diagnostic concordance rate was confirmed to be 75%, and our findings suggest that MCNs have a low malignancy potential when they are less than 4 cm in size, are asymptomatic and lack worrisome features on preoperative images.
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Affiliation(s)
- Ga Hee Kim
- Departments of Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea
| | - Kyu Choi
- Departments of Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea
| | - Namyoung Paik
- Departments of Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea
| | - Kyu Taek Lee
- Departments of Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea
| | - Jong Kyun Lee
- Departments of Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea
| | - Kwang Hyuck Lee
- Departments of Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea
| | - In Woong Han
- Departments of Surgery, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea
| | - Soo Hoon Kang
- Departments of Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea
| | - Jin Seok Heo
- Departments of Surgery, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea
| | - Joo Kyung Park
- Departments of Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea
- Department of Health Sciences and Technology, SAIHST, Sungkyunkwan University, Seoul, Korea
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27
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Ohno E, Ishikawa T, Mizutani Y, Iida T, Uetsuki K, Yashika J, Yamada K, Gibo N, Aoki T, Kawashima H. Factors associated with misdiagnosis of preoperative endoscopic ultrasound in patients with pancreatic cystic neoplasms undergoing surgical resection. J Med Ultrason (2001) 2022; 49:433-441. [PMID: 35411413 DOI: 10.1007/s10396-022-01205-7] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/27/2021] [Accepted: 02/24/2022] [Indexed: 11/29/2022]
Abstract
PURPOSE Pancreatic cystic lesions (PCLs) include various types of cysts. Accurate preoperative diagnosis is essential to avoid unnecessary surgery on benign cysts. In this study, we aimed to identify the factors associated with misdiagnosis in preoperative endoscopic ultrasound (EUS) imaging and clinicopathological findings for PCLs. METHODS This study included 302 surgically resected patients with PCLs who underwent preoperative EUS. The preoperative EUS findings were re-evaluated and compared with the pathological diagnosis. Additionally, the factors associated with misdiagnosis of PCLs were investigated by multivariate analysis. RESULTS The preoperative diagnoses of PCLs were intraductal papillary mucinous neoplasm (IPMN) in 213 patients (70.5%), mucinous cystic neoplasm (MCN) in 33 patients (10.9%), serous neoplasm (SN) in 7 patients (2.3%), cystic degeneration cyst of solid tumor in 46 patients (15.2%), and pancreatic cancer with cystic degeneration in three patients (1.0%). A discrepancy between preoperative and postoperative diagnosis was found in 47 patients (15.6%). Based on the pathological diagnosis, the sensitivity of preoperative EUS imaging was IPMN 97.6% (206/211), MCN 90.0% (18/19), cystic degeneration 87.1% (27/31), and SN 15.4% (2/13). Multivariate analysis revealed that main pancreatic duct (MPD) communication ( - ) (odds ratio (OR), 4.54; 95% confidence interval (CI) 1.29-15.9), honeycomb-like structure ( +) (OR, 14.7; 95% CI 2.61-83.3), and MPD size ≦ 2 mm (OR, 16.3; 95% CI 3.93-67.6) were independently associated with misdiagnosis. CONCLUSION For cases in which MCN and cystic degeneration of solid tumor are presumed based on preoperative EUS imaging and cases with PCLs with a honeycomb-like structure, diagnosis with multimodalities or fluid analysis with EUS-guided fine-needle aspiration should be considered.
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Affiliation(s)
- Eizaburo Ohno
- Department of Gastroenterology and Hepatology, Nagoya University Graduate School of Medicine, Nagoya University Hospital, 65 Tsurumai-cho, Showa-ku, Nagoya, Aichi, 466-8550, Japan.
| | - Takuya Ishikawa
- Department of Gastroenterology and Hepatology, Nagoya University Graduate School of Medicine, Nagoya University Hospital, 65 Tsurumai-cho, Showa-ku, Nagoya, Aichi, 466-8550, Japan
| | - Yasuyuki Mizutani
- Department of Gastroenterology and Hepatology, Nagoya University Graduate School of Medicine, Nagoya University Hospital, 65 Tsurumai-cho, Showa-ku, Nagoya, Aichi, 466-8550, Japan
| | - Tadashi Iida
- Department of Gastroenterology and Hepatology, Nagoya University Graduate School of Medicine, Nagoya University Hospital, 65 Tsurumai-cho, Showa-ku, Nagoya, Aichi, 466-8550, Japan
| | - Kota Uetsuki
- Department of Gastroenterology and Hepatology, Nagoya University Graduate School of Medicine, Nagoya University Hospital, 65 Tsurumai-cho, Showa-ku, Nagoya, Aichi, 466-8550, Japan
| | - Jun Yashika
- Department of Gastroenterology and Hepatology, Nagoya University Graduate School of Medicine, Nagoya University Hospital, 65 Tsurumai-cho, Showa-ku, Nagoya, Aichi, 466-8550, Japan
| | - Kenta Yamada
- Department of Endoscopy, Nagoya University Hospital, Nagoya, Aichi, Japan
| | - Noriaki Gibo
- Department of Gastroenterology and Hepatology, Nagoya University Graduate School of Medicine, Nagoya University Hospital, 65 Tsurumai-cho, Showa-ku, Nagoya, Aichi, 466-8550, Japan
| | - Toshinori Aoki
- Department of Gastroenterology and Hepatology, Nagoya University Graduate School of Medicine, Nagoya University Hospital, 65 Tsurumai-cho, Showa-ku, Nagoya, Aichi, 466-8550, Japan
| | - Hiroki Kawashima
- Department of Endoscopy, Nagoya University Hospital, Nagoya, Aichi, Japan
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28
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Ardeshna DR, Rangwani S, Cao T, Pawlik TM, Stanich PP, Krishna SG. Intraductal Papillary Mucinous Neoplasms in Hereditary Cancer Syndromes. Biomedicines 2022; 10:1475. [PMID: 35884779 PMCID: PMC9313108 DOI: 10.3390/biomedicines10071475] [Citation(s) in RCA: 11] [Impact Index Per Article: 3.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/22/2022] [Revised: 06/17/2022] [Accepted: 06/20/2022] [Indexed: 11/16/2022] Open
Abstract
Hereditary pancreatic cancer, which includes patients with familial pancreatic cancer (FPC) and hereditary pancreatic cancer syndromes, accounts for about 10% of all pancreatic cancer diagnoses. The early detection of pre-cancerous pancreatic cysts has increasingly become a focus of interest in recent years as a potential avenue to lower pancreatic cancer incidence and mortality. Intraductal papillary mucinous cystic neoplasms (IPMNs) are recognized precursor lesions of pancreatic cancer. IPMNs have high prevalence in patients with hereditary pancreatic cancer and their relatives. While various somatic mutations have been identified in IPMNs, certain germline mutations associated with hereditary cancer syndromes have also been identified in IPMNs, suggesting a role in their formation. While the significance for the higher prevalence of IPMNs or similar germline mutations in these high-risk patients remain unclear, IPMNs do represent pre-malignant lesions that need close surveillance. This review summarizes the available literature on the incidence and prevalence of IPMNs in inherited genetic predisposition syndromes and FPC and speculates if IPMN and pancreatic cancer surveillance in these high-risk individuals needs to change.
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Affiliation(s)
- Devarshi R. Ardeshna
- Department of Internal Medicine, Ohio State University Wexner Medical Center, Columbus, OH 43210, USA; (D.R.A.); (S.R.)
| | - Shiva Rangwani
- Department of Internal Medicine, Ohio State University Wexner Medical Center, Columbus, OH 43210, USA; (D.R.A.); (S.R.)
| | - Troy Cao
- College of Medicine, Ohio State University, Columbus, OH 43210, USA;
| | - Timothy M. Pawlik
- Department of Surgery, The Ohio State University Wexner Medical Center, Columbus, OH 43210, USA;
| | - Peter P. Stanich
- Division of Gastroenterology, Hepatology and Nutrition, Department of Internal Medicine, The Ohio State University Wexner Medical Center, Columbus, OH 43210, USA;
| | - Somashekar G. Krishna
- Division of Gastroenterology, Hepatology and Nutrition, Department of Internal Medicine, The Ohio State University Wexner Medical Center, Columbus, OH 43210, USA;
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29
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Fukumura Y, Kinowaki Y, Matsuda Y, Takase M, Tonosaki M, Minagawa M, Saiura A, Tanabe M, Okano K, Suzuki Y, Kato K, Yao T. Intralobular distribution of ovarian-like stroma in pancreatic mucinous cystic neoplasms: a discussion on its tumorigenesis. Sci Rep 2022; 12:3326. [PMID: 35228647 PMCID: PMC8885835 DOI: 10.1038/s41598-022-07416-9] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/16/2021] [Accepted: 02/17/2022] [Indexed: 11/09/2022] Open
Abstract
AbstractPancreatic mucinous cystic neoplasm (MCN) has two histological components: tumor epithelia and ovarian-like stroma (OLS). To examine the progression and changes in pancreatic MCNs, we analyzed the distribution, amount, immunohistochemical phenotype, presence of theca cells of OLS, and tumor epithelium in 45 surgically resected MCN cases, comparing them with tumor sizes. The OLS data of female MCN cases were also compared between those who were ≤ 51 years old and those > 51 years old to see the effect of menopause on MCN histology. Non-mucinous type epithelium was present in all low-grade MCNs, but its ratio decreased with tumor size (p < 0.001), suggesting that epithelial mucinous changes are a progression phenomenon. The intralobular distribution of OLS was observed in 28.8% of MCN cases and was related to smaller tumor size (p < 0.0001), suggesting intralobular involvement of early MCNs. The nuclear expression of β-catenin and the cytoplasmic expression of α-smooth muscle actin (SMA) was observed in almost all OLS. OLS tended to be lesser among female cases aged > 51 years than those ≤ 51 years old, however it did not reach statistical significance. This is the first study to show the intralobular distribution of OLS.
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30
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Sakihama K, Koga Y, Yamamoto T, Shimada Y, Yamada Y, Kawata J, Shindo K, Nakamura M, Oda Y. RNF43 as a predictor of malignant transformation of pancreatic mucinous cystic neoplasm. Virchows Arch 2022; 480:1189-1199. [PMID: 35066614 DOI: 10.1007/s00428-022-03277-9] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/16/2021] [Revised: 12/23/2021] [Accepted: 01/12/2022] [Indexed: 11/26/2022]
Abstract
Mucinous cystic neoplasm (MCN) of the pancreas rarely progresses to invasive carcinoma, but few studies have analyzed genomic alterations involved in its malignant transformation. The relationships of ring finger protein 43 (RNF43) mutations with cytological atypia, RNF43 protein expression, and Wnt signaling proteins in MCN remain unclear. This study included 106 MCN cases, classified into 89 low-grade dysplasia (LG), 9 high-grade dysplasia (HG), and 8 invasive carcinoma (INV). We analyzed HG/INV and LG lesions of 9 HG/INV cases and LG lesions of 9 LG cases using targeted sequencing and confirmed the protein expression of RNF43 and β-catenin. The frequency of RNF43 mutations was significantly higher in HG/INV cases than in LG cases. Furthermore, HG/INV lesions (56%) and LG lesions (33%) of HG/INV cases possessed RNF43 mutation, whereas no such mutation was detected in any LG cases. The expression of RNF43 was reduced in 71% of HG/INV cases and significantly correlated with histological grade and aberrant expression of β-catenin. In 3 of 5 RNF43-mutated cases, the expression of RNF43 was reduced, but there was no significant correlation between RNF43 mutation and protein expression. MCNs frequently harbored KRAS mutations, at rates of 100% in HG/INV lesions and 50% in LG lesions of HG/INV and LG cases. There was no significant difference in mutation frequency in LG lesions between HG/INV and LG cases. These results suggest that RNF43 mutations may be involved in and predictive of malignant transformation from an early stage of MCN.
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Affiliation(s)
- Kukiko Sakihama
- Department of Anatomic Pathology, Graduate School of Medical Sciences, Kyushu University, 3-1-1 Maidashi, Higashiku, Fukuoka, 812-8582, Japan
- Department of Surgery and Oncology, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan
| | - Yutaka Koga
- Department of Pathology, National Hospital Organization Kyushu Cancer Center, Fukuoka, Japan
| | - Takeo Yamamoto
- Department of Anatomic Pathology, Graduate School of Medical Sciences, Kyushu University, 3-1-1 Maidashi, Higashiku, Fukuoka, 812-8582, Japan
- Department of Surgery and Oncology, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan
| | - Yuki Shimada
- Department of Anatomic Pathology, Graduate School of Medical Sciences, Kyushu University, 3-1-1 Maidashi, Higashiku, Fukuoka, 812-8582, Japan
- Department of Surgery and Oncology, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan
| | - Yutaka Yamada
- Department of Anatomic Pathology, Graduate School of Medical Sciences, Kyushu University, 3-1-1 Maidashi, Higashiku, Fukuoka, 812-8582, Japan
- Department of Surgery and Oncology, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan
| | - Jun Kawata
- Department of Anatomic Pathology, Graduate School of Medical Sciences, Kyushu University, 3-1-1 Maidashi, Higashiku, Fukuoka, 812-8582, Japan
- Department of Surgery and Oncology, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan
| | - Koji Shindo
- Department of Surgery and Oncology, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan
| | - Masafumi Nakamura
- Department of Surgery and Oncology, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan
| | - Yoshinao Oda
- Department of Anatomic Pathology, Graduate School of Medical Sciences, Kyushu University, 3-1-1 Maidashi, Higashiku, Fukuoka, 812-8582, Japan.
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Chon HK, Moon SH, Park SW, Paik WH, Paik CN, Son BK, Song TJ, Ahn DW, Lee ES, Lee YN, Lee YS, Lee JM, Jeon TJ, Park CH, Cho KB, Lee DW. Current trends in the management of pancreatic cystic neoplasms in Korea: a national survey. Korean J Intern Med 2022; 37:63-72. [PMID: 33045809 PMCID: PMC8747915 DOI: 10.3904/kjim.2020.452] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/24/2020] [Revised: 09/22/2020] [Accepted: 09/23/2020] [Indexed: 12/03/2022] Open
Abstract
BACKGROUND/AIMS The study aimed to investigate the current practice patterns in the management of pancreatic cystic neoplasms in Korea. METHODS An electronic survey was systematically distributed by email to members of the Korean Pancreatobiliary Association from December 2019 to February 2020. RESULTS In total, 115 (110 gastroenterologists, five surgeons) completed the survey, 72.2% of whom worked in a tertiary/academic medical center. Most (65.2%) followed the 2012/2017 International Association of Pancreatology guidelines for the management of pancreatic cystic neoplasms. A gadolinium-enhanced magnetic resonance imaging/magnetic resonance cholangiopancreatography was the most common first-line diagnostic modality (42.1%), but a contrast-enhanced computed tomography scan was preferred as a subsequent surveillance tool (58.3%). Seventy-four percent of respondents routinely performed endoscopic ultrasound-guided fine needle aspiration for pancreatic cystic neoplasms with suspicious mural nodules. Endoscopic ultrasound-guided fine needle aspiration cytology (94.8%) and cystic fluid carcinoembryonic antigen (95.7%) were used for cystic fluid analysis. Most (94%) typically recommended surgery in patients with high-risk stigmata, but 18.3% also considered proceeding with surgery in patients with worrisome features. Most (96.5%) would continue surveillance of pancreatic cystic neoplasms for more than 5 years. CONCLUSION According to this survey, there was variability in the management of pancreatic cystic neoplasms among the respondents. These results suggest that the development of evidence-based guidelines for pancreatic cystic neoplasms that fit the Korean practice is needed to create an optimal approach to the management of pancreatic cystic neoplasms.
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Affiliation(s)
- Hyung Ku Chon
- Department of Internal Medicine, Institute of Wonkwang Medical Science, Wonkwang University College of Medicine, Iksan, Korea
| | - Sung Hoon Moon
- Department of Internal Medicine, Hallym University College of Medicine, Anyang, Korea
| | - Sang Wook Park
- Department of Internal Medicine, Kwangju Christian Hospital, Gwangju, Korea
| | - Woo Hyun Paik
- Department of Internal Medicine, Seoul National University College of Medicine, Seoul, Korea
| | - Chang Nyol Paik
- Department of Internal Medicine, St. Vincent's Hospital, College of Medicine, The Catholic University of Korea, Suwon, Korea
| | - Byoung Kwan Son
- Department of Internal Medicine, Eulji University College of Medicine, Seoul, Korea
| | - Tae Jun Song
- Department of Internal Medicine, University of Ulsan College of Medicine, Seoul, Korea
| | - Dong Won Ahn
- Department of Internal Medicine, Seoul National University Boramae Medical Center, Seoul, Korea
| | - Eaum Seok Lee
- Department of Internal Medicine, Chungnam National University College of Medicine, Daejeon, Korea
| | - Yun Nah Lee
- Department of Internal Medicine, Soonchunhyang University School of Medicine, Bucheon, Korea
| | - Yoon Suk Lee
- Department of Internal Medicine, Inje University Ilsan Paik Hospital, Goyang, Korea
| | - Jae Min Lee
- Department of Internal Medicine, Korea University College of Medicine, Seoul, Korea
| | - Tae Joo Jeon
- Department of Internal Medicine, Inje University Sanggye Paik Hospital, Seoul, Korea
| | - Chang Hwan Park
- Department of Internal Medicine, Chonnam National University Medical School, Gwangju, Korea
| | - Kwang Bum Cho
- Department of Internal Medicine, Keimyung University Dongsan Medical Center, Keimyung University School of Medicine, Daegu, Korea
| | - Dong Wook Lee
- Department of Internal Medicine, Daegu Catholic University School of Medicine, Daegu, Korea
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Yu TY, Zhang JS, Chen K, Yu AJ. Mucinous cystic neoplasm of the liver: A case report. World J Clin Cases 2021; 9:11475-11481. [PMID: 35071580 PMCID: PMC8717498 DOI: 10.12998/wjcc.v9.i36.11475] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/10/2021] [Revised: 09/22/2021] [Accepted: 11/15/2021] [Indexed: 02/06/2023] Open
Abstract
BACKGROUND Mucinous cystic neoplasm of the liver (MCN-L) is a cyst-forming epithelial neoplasm. The most distinguishing feature is the ovarian-type subepithelial stroma on pathological examination.
CASE SUMMARY An abdominal ultrasound incidentally revealed a liver tumor in a 32-year-old woman. Physical and laboratory examination results did not reveal any abnormalities. Enhanced abdominal computed tomography (CT) revealed a cystic space measuring 7.2 cm × 5.4 cm in the liver. Subsequent CT showed an increase in tumor size. Thus, we performed surgical resection of the tumor and gallbladder. Postoperative histopathological examination confirmed the diagnosis of MCN-L. At the 6-mo of follow-up, no recurrence was observed on ultrasound or CT.
CONCLUSION Since preoperative diagnosis of MCN-L is difficult, active surgery is recommended and helpful for the diagnosis and treatment of MCN-L.
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Affiliation(s)
- Tian-Yang Yu
- First Department of General Surgery, Affiliated Hospital of Chengde Medical University, Chengde 067000, Hebei Province, China
| | - Jing-Song Zhang
- First Department of General Surgery, Affiliated Hospital of Chengde Medical University, Chengde 067000, Hebei Province, China
| | - Kai Chen
- First Department of General Surgery, Affiliated Hospital of Chengde Medical University, Chengde 067000, Hebei Province, China
| | - Ai-Jun Yu
- First Department of General Surgery, Affiliated Hospital of Chengde Medical University, Chengde 067000, Hebei Province, China
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Robotic distal pancreatectomy using a medial approach for bulky mucinous cystic neoplasm of the pancreas (with video). Asian J Surg 2021; 45:542-543. [PMID: 34649801 DOI: 10.1016/j.asjsur.2021.09.009] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/19/2021] [Accepted: 09/23/2021] [Indexed: 11/22/2022] Open
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Buerlein RCD, Shami VM. Management of pancreatic cysts and guidelines: what the gastroenterologist needs to know. Ther Adv Gastrointest Endosc 2021; 14:26317745211045769. [PMID: 34589706 PMCID: PMC8474323 DOI: 10.1177/26317745211045769] [Citation(s) in RCA: 4] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/04/2021] [Accepted: 08/25/2021] [Indexed: 12/15/2022] Open
Abstract
The prevalence of pancreatic cysts has increased significantly over the last
decade, partly secondary to increased quality and frequency of cross-sectional
imaging. While the majority never progress to cancer, a small number will and
need to be followed. The management of pancreatic cysts can be both confusing
and intimidating due to the multiple guidelines with varying recommendations.
Despite the differences in the specifics of the guidelines, they all agree on
several high-risk features that should get the attention of any clinician when
assessing a pancreatic cyst: presence of a mural nodule or solid component,
dilation of the main pancreatic duct (or presence of main duct intraductal
papillary mucinous neoplasm), pancreatic cyst size ⩾3–4 cm, or positive cytology
on pancreatic cyst fluid aspiration. Other important criteria to consider
include rapid cyst growth (⩾5 mm/year), elevated serum carbohydrate antigen 19-9
levels, new-onset diabetes mellitus, or acute pancreatitis thought to be related
to the cystic lesion.
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Affiliation(s)
| | - Vanessa M Shami
- University of Virginia Digestive Health, 1215 Lee Street, Charlottesville, VA 22903, USA
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35
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Sometimes You Have to Judge a Book by Its Cover: The Case of a Masquerading Pancreatic Mucinous Cyst. ACG Case Rep J 2021; 8:e00554. [PMID: 34549054 PMCID: PMC8443815 DOI: 10.14309/crj.0000000000000554] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/23/2020] [Accepted: 10/04/2020] [Indexed: 11/17/2022] Open
Abstract
Pancreatic cystic lesions are difficult to evaluate amid acute pancreatitis. Without previous pancreatic imaging, it is challenging to discern between pancreatic acute fluid collections and cystic neoplasms. We present a 29-year-old woman with acute pancreatitis and initial cross-sectional imaging suggesting a 2.8-cm cystic lesion in the body/tail of the pancreas. Endoscopic ultrasound completed 5 weeks after index presentation revealed findings worrisome for a cystic neoplasm, but fine-needle aspiration findings suggested lesion to be a pseudocyst (normal carcinoembryonic antigen and cytology, negative mucin stain).
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36
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37
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Guo N, Ning H, Xing B, Ma Y. Mucinous cystic neoplasm of the pancreas misdiagnosed as pancreatic pseudocyst: A case report. Asian J Surg 2021; 44:1602-1603. [PMID: 34503875 DOI: 10.1016/j.asjsur.2021.08.038] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/10/2021] [Revised: 08/15/2021] [Accepted: 08/20/2021] [Indexed: 11/17/2022] Open
Affiliation(s)
- Na Guo
- First Clinical Medical School, Lanzhou University, Lanzhou Gansu, 730000, China
| | - Haihui Ning
- First Clinical Medical School, Lanzhou University, Lanzhou Gansu, 730000, China
| | - Bomin Xing
- First Clinical Medical School, Lanzhou University, Lanzhou Gansu, 730000, China
| | - Yuqing Ma
- First Hospital of Lanzhou University, Lanzhou Gansu, 730000, China.
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38
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Liang H, Xie W, Lin X, Wang T, Xie J, Wang C, Xiao S, Guo Y. Pathologic T1 and T2 encapsulated invasive carcinomas arising from mucinous cystic neoplasms of the pancreas have favorable prognosis and might be treated conservatively. J Pathol Clin Res 2021; 7:507-516. [PMID: 34062050 PMCID: PMC8363923 DOI: 10.1002/cjp2.225] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/22/2021] [Revised: 03/22/2021] [Accepted: 04/22/2021] [Indexed: 12/28/2022]
Abstract
Carcinoma arising from a mucinous cystic neoplasm (MCN) of the pancreas is termed MCN with associated invasive carcinoma (MCN-AIC) in the fifth WHO classification of digestive tumors (2019). The prognosis of this malignancy varies depending on the relationship of the invasive carcinoma to the cyst capsule, but limited data are available. This study identified 165 surgically resected MCNs including 15 MCN-AICs from a single center between 2008 and 2018 and analyzed their clinicopathologic features. The results confirmed that non-invasive MCNs were completely cured by surgery. All MCN-AICs showing an encapsulated invasion pattern (defined as invasive carcinoma limited to the ovarian-type stroma, cystic septa, and capsule) had an excellent prognosis with a 5-year survival rate of 100%, even when the size of the invasive component was up to stage T2. By contrast, MCN-AICs with extracapsular involvement had unfavorable clinical outcomes. Our study demonstrates that the pattern of invasion of MCN-AIC can predict patient prognosis. Pathologic stage T1 and T2 encapsulated MCN-AICs may be completely cured with surgical resection alone or when combined with postoperative chemotherapy.
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MESH Headings
- Adult
- Female
- Humans
- Male
- Middle Aged
- Neoplasm Grading
- Neoplasm Invasiveness/diagnostic imaging
- Neoplasm Invasiveness/pathology
- Neoplasms, Cystic, Mucinous, and Serous/diagnosis
- Neoplasms, Cystic, Mucinous, and Serous/diagnostic imaging
- Neoplasms, Cystic, Mucinous, and Serous/pathology
- Neoplasms, Cystic, Mucinous, and Serous/surgery
- Pancreas/diagnostic imaging
- Pancreas/pathology
- Pancreas/surgery
- Pancreatectomy
- Prognosis
- Survival Rate
- Tomography, X-Ray Computed
- Treatment Outcome
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Affiliation(s)
- Huaiyu Liang
- Department of PathologyRuijin Hospital, Shanghai Jiao Tong University School of MedicineShanghaiPR China
| | - Wen Xie
- Department of PathologyRuijin Hospital, Shanghai Jiao Tong University School of MedicineShanghaiPR China
- Department of PathologyZhong'nan Hospital of Wuhan UniversityWuhanPR China
| | - Xiaozhu Lin
- Department of PathologyRuijin Hospital, Shanghai Jiao Tong University School of MedicineShanghaiPR China
- Department of RadiologyRuijin Hospital, Shanghai Jiao Tong University School of MedicineShanghaiPR China
| | - Ting Wang
- Department of PathologyRuijin Hospital, Shanghai Jiao Tong University School of MedicineShanghaiPR China
| | - Jing Xie
- Department of PathologyRuijin Hospital, Shanghai Jiao Tong University School of MedicineShanghaiPR China
| | - Chaofu Wang
- Department of PathologyRuijin Hospital, Shanghai Jiao Tong University School of MedicineShanghaiPR China
| | - Shu‐Yuan Xiao
- Department of PathologyZhong'nan Hospital of Wuhan UniversityWuhanPR China
- Department of PathologyUniversity of Chicago MedicineChicagoILUSA
| | - Yan Guo
- Department of PathologyRuijin Hospital, Shanghai Jiao Tong University School of MedicineShanghaiPR China
- Histo Pathology Diagnostic CenterShanghaiPR China
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Liu S, Zhang Z, Guo C, Yu Z, He S, Khan J, Jiang B, Song Y, Peng C. Obstructive Jaundice Caused by Mucinous Cystic Tumor of Gallbladder: A Case Report and Literature Review. J Clin Transl Hepatol 2021; 9:598-602. [PMID: 34447691 PMCID: PMC8369014 DOI: 10.14218/jcth.2020.00123] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/18/2020] [Revised: 01/03/2021] [Accepted: 03/19/2021] [Indexed: 12/14/2022] Open
Abstract
Mucinous cystic tumor of the gallbladder is an extremely rare benign tumor, with potential for malignant degeneration. Mucinous cystic tumors of the cystic duct are divided into mucinous cystadenoma and mucinous cystadenocarcinoma. Currently, cystadenoma is generally considered to be a precancerous lesion of cystadenocarcinoma. At present, there are few cases reported worldwide, and there are no relevant guidelines for diagnosis and treatment of this disease. This article presents the collected clinical data of a patient with mucinous cystic tumor of the gallbladder who was admitted to the First Affiliated Hospital of Hunan Normal University, with the characteristics of the disease summarized in combination with a focused literature review.
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Affiliation(s)
- Sulai Liu
- Department of Hepatobiliary Surgery/Hunan Research Center of Biliary Disease, Hunan Provincial People’s Hospital/The First Affiliated Hospital of Hunan Normal University, Changsha, Hunan, China
- Biliary Disease Research Laboratory of Hunan Provincial People’s Hospital, Key Laboratory of Hunan Normal University, Changsha, Hunan, China
- Clinical Medical Technology Research Center of Hunan Provincial for Biliary Disease Prevention and Treatment, Changsha, Hunan, China
| | - Zhihua Zhang
- Department of Hepatobiliary Surgery/Hunan Research Center of Biliary Disease, Hunan Provincial People’s Hospital/The First Affiliated Hospital of Hunan Normal University, Changsha, Hunan, China
- Biliary Disease Research Laboratory of Hunan Provincial People’s Hospital, Key Laboratory of Hunan Normal University, Changsha, Hunan, China
- Clinical Medical Technology Research Center of Hunan Provincial for Biliary Disease Prevention and Treatment, Changsha, Hunan, China
| | - Chao Guo
- Department of Hepatobiliary Surgery/Hunan Research Center of Biliary Disease, Hunan Provincial People’s Hospital/The First Affiliated Hospital of Hunan Normal University, Changsha, Hunan, China
- Biliary Disease Research Laboratory of Hunan Provincial People’s Hospital, Key Laboratory of Hunan Normal University, Changsha, Hunan, China
- Clinical Medical Technology Research Center of Hunan Provincial for Biliary Disease Prevention and Treatment, Changsha, Hunan, China
| | - Zhangtao Yu
- Department of Hepatobiliary Surgery/Hunan Research Center of Biliary Disease, Hunan Provincial People’s Hospital/The First Affiliated Hospital of Hunan Normal University, Changsha, Hunan, China
- Biliary Disease Research Laboratory of Hunan Provincial People’s Hospital, Key Laboratory of Hunan Normal University, Changsha, Hunan, China
- Clinical Medical Technology Research Center of Hunan Provincial for Biliary Disease Prevention and Treatment, Changsha, Hunan, China
| | - Siyuan He
- Department of Hepatobiliary Surgery/Hunan Research Center of Biliary Disease, Hunan Provincial People’s Hospital/The First Affiliated Hospital of Hunan Normal University, Changsha, Hunan, China
- Biliary Disease Research Laboratory of Hunan Provincial People’s Hospital, Key Laboratory of Hunan Normal University, Changsha, Hunan, China
- Clinical Medical Technology Research Center of Hunan Provincial for Biliary Disease Prevention and Treatment, Changsha, Hunan, China
| | - Junaid Khan
- Department of Hepatobiliary Surgery/Hunan Research Center of Biliary Disease, Hunan Provincial People’s Hospital/The First Affiliated Hospital of Hunan Normal University, Changsha, Hunan, China
- Biliary Disease Research Laboratory of Hunan Provincial People’s Hospital, Key Laboratory of Hunan Normal University, Changsha, Hunan, China
- Clinical Medical Technology Research Center of Hunan Provincial for Biliary Disease Prevention and Treatment, Changsha, Hunan, China
| | - Bo Jiang
- Department of Hepatobiliary Surgery/Hunan Research Center of Biliary Disease, Hunan Provincial People’s Hospital/The First Affiliated Hospital of Hunan Normal University, Changsha, Hunan, China
- Biliary Disease Research Laboratory of Hunan Provincial People’s Hospital, Key Laboratory of Hunan Normal University, Changsha, Hunan, China
- Clinical Medical Technology Research Center of Hunan Provincial for Biliary Disease Prevention and Treatment, Changsha, Hunan, China
| | - Yinghui Song
- Department of Hepatobiliary Surgery/Hunan Research Center of Biliary Disease, Hunan Provincial People’s Hospital/The First Affiliated Hospital of Hunan Normal University, Changsha, Hunan, China
- Biliary Disease Research Laboratory of Hunan Provincial People’s Hospital, Key Laboratory of Hunan Normal University, Changsha, Hunan, China
- Clinical Medical Technology Research Center of Hunan Provincial for Biliary Disease Prevention and Treatment, Changsha, Hunan, China
- Correspondence to: Yinghui Song and Chuang Peng, Department of Hepatobiliary Surgery, Hunan Provincial People’s Hospital/The First Affiliated Hospital of Hunan Normal University, Changsha, Hunan 410004, China. Tel: +86-731-83929520, Fax: +86-731-83929520, E-mail: (YS) or (CP)
| | - Chuang Peng
- Department of Hepatobiliary Surgery/Hunan Research Center of Biliary Disease, Hunan Provincial People’s Hospital/The First Affiliated Hospital of Hunan Normal University, Changsha, Hunan, China
- Biliary Disease Research Laboratory of Hunan Provincial People’s Hospital, Key Laboratory of Hunan Normal University, Changsha, Hunan, China
- Clinical Medical Technology Research Center of Hunan Provincial for Biliary Disease Prevention and Treatment, Changsha, Hunan, China
- Correspondence to: Yinghui Song and Chuang Peng, Department of Hepatobiliary Surgery, Hunan Provincial People’s Hospital/The First Affiliated Hospital of Hunan Normal University, Changsha, Hunan 410004, China. Tel: +86-731-83929520, Fax: +86-731-83929520, E-mail: (YS) or (CP)
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Vullierme MP, Gregory J, Rebours V, Cros J, Abelhady-Attia Y, Vilgrain V, Aguilera-Munoz L, Laurent L, Levy P, Sauvanet A, Ronot M. MRI is useful to suggest and exclude malignancy in mucinous cystic neoplasms of the pancreas. Eur Radiol 2021; 32:1297-1307. [PMID: 34374801 DOI: 10.1007/s00330-021-08091-6] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/06/2021] [Accepted: 05/21/2021] [Indexed: 12/15/2022]
Abstract
OBJECTIVES To evaluate the value of MRI in differentiating benign (b-MCN) and malignant (m-MCN) MCN. European guidelines suggest that certain mucinous cystic neoplasms (MCN) of the pancreas can be conservatively managed. METHODS A retrospective single-center study of consecutive patients with resected MCN. MRIs were independently reviewed by two readers blinded to the pathological results. The authors compared b-MCN (i.e., mucinous-cystadenoma comprising high-grade dysplasia (HGD)) and m-MCN (i.e., cystadenocarcinoma). RESULTS Sixty-three patients (62 women [98%]) with 63 MCN (6 m-MCN, 2 HGD) were included. m-MCN tumors had a tendency to be larger than b-MCN (median 86 [25-103] vs. 45 [17-130] mm, p = .055). The combination of signal heterogeneity on T2-weighted imaging, wall thickness ≥ 5 mm, the presence of mural nodules ≥ 9 mm, and enhancing septa had an area under the ROC curve of 0.97 (95% CI 0.91-1.00) for the diagnosis of m-MCN. A total of 24 (37%), 20 (32%), 10 (16%), 5 (8%), and 4 (6%) out of 63 MCNs showed 0, 1, 2, 3, and 4 of these features, respectively. The corresponding rate of m-MCN was 0%, 0%, 10%, 20%, and 100%, respectively, with a good-to-excellent inter-reader agreement. Patterns with a high NPV for m-MCN included an absence of enhancing septa or walls (NPV 97% and 100%, respectively), wall thickness < 3 mm (NPV 100%), and no mural nodules (NPV 100%). CONCLUSIONS A combination of 4 imaging features suggests malignant MCN on MRI. On the other hand, visualization of a thin non-enhancing wall with no mural nodules suggests benign MCN. KEY POINTS • A heterogenous signal on T2-weighted MRI, a ≥ 5-mm-thick wall, mural nodules ≥ 9 mm, and/or enhancing septa suggest malignant MCNs. • A thin non-enhancing wall with no mural nodules suggests benign MCNs. • MRI should be performed in the pre-therapeutic evaluation of MCN to help determine the therapeutic strategy in these patients.
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Affiliation(s)
- Marie-Pierre Vullierme
- Assistance Publique-Hôpitaux de Paris, APHP.Nord, Department of Radiology, Beaujon University Hospital, 100 bd general Leclerc, 92110, Clichy, France. .,Université Paris Diderot-Paris 7, Université de Paris, F-75018, Paris, France.
| | - Jules Gregory
- Assistance Publique-Hôpitaux de Paris, APHP.Nord, Department of Radiology, Beaujon University Hospital, 100 bd general Leclerc, 92110, Clichy, France
| | - Vinciane Rebours
- Université Paris Diderot-Paris 7, Université de Paris, F-75018, Paris, France.,Assistance Publique-Hôpitaux de Paris, APHP.Nord, Department of Pancreatology, Beaujon Hospital, 100 bd general Leclerc, 92110, Clichy, France
| | - Jerome Cros
- Université Paris Diderot-Paris 7, Université de Paris, F-75018, Paris, France.,Assistance Publique-Hôpitaux de Paris, APHP.Nord, Department of Pathology, Beaujon University Hospital, 100 bd general Leclerc, 92110, Clichy, France
| | - Yasser Abelhady-Attia
- Department of Radiology, Benha University Hospital, Fareed Nada Street, Benha, Qalubiya Governorate, 13511, Egypt
| | - Valerie Vilgrain
- Assistance Publique-Hôpitaux de Paris, APHP.Nord, Department of Radiology, Beaujon University Hospital, 100 bd general Leclerc, 92110, Clichy, France.,Centre de Recherche de l'Inflammation (CRI), INSERM U1149, Université de Paris, F-75018, Paris, France
| | - Lina Aguilera-Munoz
- Assistance Publique-Hôpitaux de Paris, APHP.Nord, Department of Pancreatology, Beaujon Hospital, 100 bd general Leclerc, 92110, Clichy, France
| | - Lucie Laurent
- Assistance Publique-Hôpitaux de Paris, APHP.Nord, Department of Pancreatology, Beaujon Hospital, 100 bd general Leclerc, 92110, Clichy, France
| | - Philippe Levy
- Université Paris Diderot-Paris 7, Université de Paris, F-75018, Paris, France.,Assistance Publique-Hôpitaux de Paris, APHP.Nord, Department of Pancreatology, Beaujon Hospital, 100 bd general Leclerc, 92110, Clichy, France
| | - Alain Sauvanet
- Université Paris Diderot-Paris 7, Université de Paris, F-75018, Paris, France.,Assistance Publique-Hôpitaux de Paris, APHP.Nord, Department of Hepato Pancreato Biliary Surgery, Beaujon University Hospital, 100 bd general Leclerc, 92110, Clichy, France
| | - Maxime Ronot
- Assistance Publique-Hôpitaux de Paris, APHP.Nord, Department of Radiology, Beaujon University Hospital, 100 bd general Leclerc, 92110, Clichy, France.,Université Paris Diderot-Paris 7, Université de Paris, F-75018, Paris, France
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41
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Sato H, Liss AS, Mizukami Y. Large-duct pattern invasive adenocarcinoma of the pancreas-a variant mimicking pancreatic cystic neoplasms: A minireview. World J Gastroenterol 2021; 27:3262-3278. [PMID: 34163110 PMCID: PMC8218369 DOI: 10.3748/wjg.v27.i23.3262] [Citation(s) in RCA: 10] [Impact Index Per Article: 2.5] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/28/2021] [Revised: 03/09/2021] [Accepted: 05/17/2021] [Indexed: 02/06/2023] Open
Abstract
Pancreatic cancer currently has no subtypes that inform clinical decisions; hence, there exists an opportunity to rearrange the morphological and molecular taxonomy that guides a better understanding of tumor characteristics. Nonetheless, accumulating studies to date have revealed the large-duct type variant, a unique subtype of pancreatic ductal adenocarcinoma (PDA) with cystic features. This subtype often radiographically mimics intraductal papillary mucinous neoplasms (IPMNs) and involves multiple small cysts occasionally associated with solid masses. The "bunch-of-grapes" sign, an imaging characteristic of IPMNs, is absent in large-duct PDA. Large-duct PDA defines the mucin profile, and genetic alterations are useful in distinguishing large-duct PDA from IPMNs. Histologically, neoplastic ducts measure over 0.5 mm, forming large ductal elements. Similar to classic PDAs, this subtype is frequently accompanied by perineural invasion and abundant desmoplastic reactions, and KRAS mutations in codon 12 are nearly ubiquitous. Despite such morphological similarities with IPMNs, the prognosis of large-duct PDA is equivalent to that of classic PDA. Differential diagnosis is therefore essential.
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Affiliation(s)
- Hiroki Sato
- Division of Gastroenterology and Hematology/Oncology, Department of Medicine, Asahikawa Medical University, Asahikawa 0788510, Hokkaido, Japan
- Department of Surgery, Massachusetts General Hospital and Harvard Medical School, Massachusetts General Hospital, Boston, MA 02114, United States
| | - Andrew Scott Liss
- Department of Surgery, Massachusetts General Hospital and Harvard Medical School, Massachusetts General Hospital, Boston, MA 02114, United States
| | - Yusuke Mizukami
- Division of Gastroenterology and Hematology/Oncology, Department of Medicine, Asahikawa Medical University, Asahikawa 0788510, Hokkaido, Japan
- Institute of Biomedical Research, Sapporo Higashi Tokushukai Hospital, Sapporo 0650033, Hokkaido, Japan
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42
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Farahmandi S, Elessawy M, Bauerschlag DO, Pecks U, Abdullazade S, Beckmann JH, Heilmann T, Rumpf AL, Maass N, Jansen P, Winkler V. Mucinous Cystic Neoplasm of Pancreas in a Pregnant Woman Presenting with Severe Anemia and Gastric Bleeding: Case Report and Review of the Literature. Healthcare (Basel) 2021; 9:healthcare9050540. [PMID: 34066323 PMCID: PMC8148137 DOI: 10.3390/healthcare9050540] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/16/2021] [Revised: 04/16/2021] [Accepted: 04/23/2021] [Indexed: 12/28/2022] Open
Abstract
Mucinous cystic neoplasms of the pancreas are uncommon and especially their occurrence during pregnancy is an extremely rare event which necessitates an individualized and interdisciplinary management. A 33-year old woman was referred to our department during her third trimester of pregnancy (34th week of gestation) with severe anemia and tarry stools. Based on gastroscopic findings, our interdisciplinary team suspected a gastrointestinal stromal tumor and therefore indicated a prompt delivery via cesarean section completed with an oncological resection of the neoplasm. Histological examination subsequently showed a mucinous cystic neoplasm of the pancreas with no evidence of malignancy. To review the prevalence of mucinous cystic neoplasms and to discuss diagnosis and treatment during pregnancy. Moreover, we critically value the indication of preterm delivery and the oncological procedure in the perspective of outcome for mother and infant. A bleeding gastrointestinal tumor during pregnancy represents a life-threatening risk for mother and infant and requires an immediate interdisciplinary treatment. The urgency and radicality of the therapy should be adapted according to individual findings. As our patient's tumor was suspected of having a malignant progression, an extensive surgical intervention was necessary.
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Affiliation(s)
- Susan Farahmandi
- Department of Gynecology and Obstetrics, Campus Kiel, University Medical Center UKSH, Arnold-Heller-Straße 3, Haus C, 24105 Kiel, Germany; (S.F.); (M.E.); (D.O.B.); (U.P.); (T.H.); (A.-L.R.); (N.M.); (P.J.)
| | - Mohamed Elessawy
- Department of Gynecology and Obstetrics, Campus Kiel, University Medical Center UKSH, Arnold-Heller-Straße 3, Haus C, 24105 Kiel, Germany; (S.F.); (M.E.); (D.O.B.); (U.P.); (T.H.); (A.-L.R.); (N.M.); (P.J.)
| | - Dirk O. Bauerschlag
- Department of Gynecology and Obstetrics, Campus Kiel, University Medical Center UKSH, Arnold-Heller-Straße 3, Haus C, 24105 Kiel, Germany; (S.F.); (M.E.); (D.O.B.); (U.P.); (T.H.); (A.-L.R.); (N.M.); (P.J.)
| | - Ulrich Pecks
- Department of Gynecology and Obstetrics, Campus Kiel, University Medical Center UKSH, Arnold-Heller-Straße 3, Haus C, 24105 Kiel, Germany; (S.F.); (M.E.); (D.O.B.); (U.P.); (T.H.); (A.-L.R.); (N.M.); (P.J.)
| | - Samir Abdullazade
- Department of Pathology, Campus Kiel, University Medical Center UKSH, Arnold-Heller-Straße 3, Haus U33, 24105 Kiel, Germany;
| | - Jan Henrik Beckmann
- Department of General, Abdominal, Thoracic, Transplantation and Pediatric Surgery, Campus Kiel, University Medical Center UKSH, Arnold-Heller-Straße 3, Haus U33, 24105 Kiel, Germany;
| | - Thorsten Heilmann
- Department of Gynecology and Obstetrics, Campus Kiel, University Medical Center UKSH, Arnold-Heller-Straße 3, Haus C, 24105 Kiel, Germany; (S.F.); (M.E.); (D.O.B.); (U.P.); (T.H.); (A.-L.R.); (N.M.); (P.J.)
| | - Anna-Lena Rumpf
- Department of Gynecology and Obstetrics, Campus Kiel, University Medical Center UKSH, Arnold-Heller-Straße 3, Haus C, 24105 Kiel, Germany; (S.F.); (M.E.); (D.O.B.); (U.P.); (T.H.); (A.-L.R.); (N.M.); (P.J.)
| | - Nicolai Maass
- Department of Gynecology and Obstetrics, Campus Kiel, University Medical Center UKSH, Arnold-Heller-Straße 3, Haus C, 24105 Kiel, Germany; (S.F.); (M.E.); (D.O.B.); (U.P.); (T.H.); (A.-L.R.); (N.M.); (P.J.)
| | - Peer Jansen
- Department of Gynecology and Obstetrics, Campus Kiel, University Medical Center UKSH, Arnold-Heller-Straße 3, Haus C, 24105 Kiel, Germany; (S.F.); (M.E.); (D.O.B.); (U.P.); (T.H.); (A.-L.R.); (N.M.); (P.J.)
| | - Vincent Winkler
- Department of Gynecology and Obstetrics, Campus Kiel, University Medical Center UKSH, Arnold-Heller-Straße 3, Haus C, 24105 Kiel, Germany; (S.F.); (M.E.); (D.O.B.); (U.P.); (T.H.); (A.-L.R.); (N.M.); (P.J.)
- Correspondence: ; Tel.: +49-431-500-21401
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Van Treeck BJ, Horton RK, Lee HE, Rosty C, Pai RK, Graham RP. Mesenteric and Retroperitoneal Mucinous Cystic Neoplasms: A Case Series. Int J Surg Pathol 2021; 29:606-614. [PMID: 33736504 DOI: 10.1177/1066896921993536] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/17/2022]
Abstract
Aims. Mucinous cystic neoplasms (MCNs) are cystic neoplasms with mucinous epithelium surrounded by ovarian-like stroma. Extraovarian MCN occurring in the liver and pancreas have been well characterized. However, only rare case reports of MCN arising outside of these locations have been reported. MCNs arising in unusual locations should enter the differential diagnosis of mucinous intra-abdominal tumors and must be distinguished from more common mimics. Therefore, we aimed to examine a series of MCNs of the retroperitoneum and mesentery to characterize the clinicopathologic features of this entity. Methods and results. Seven MCNs arising in the abdominal mesentery or retroperitoneum were retrospectively identified. A clinicopathologic, histologic, and immunohistochemical (keratin 7, keratin 19, keratin 20, calretinin, inhibin-α, steroidogenic factor-1 (SF-1), estrogen receptor (ER), progesterone receptor (PR), PAX8, CDX2, and CD10) analysis was performed. All 7 MCNs were from females with a median age of 41 years old and a median size of 8 cm. All cases demonstrated mucinous with or without concomitant non-mucinous epithelium overlying spindle cell ovarian-like stroma. Luteinized cells were noted. The epithelium was positive for keratin 7 and keratin 19 in all 7 cases, while the stroma expressed ER, PR, and SF-1 in all cases stained. Calretinin was focally positive in the stroma of 3 of 7 cases, while inhibin-α was focally expressed in 5 of 6 cases. Conclusions. These results highlight the clinicopathologic, histologic, and immunophenotypic similarities between MCNs of the mesentery, retroperitoneum, pancreas, and liver. Overlapping features suggest a common histogenesis for all MCNs, which could include periductal fetal mesenchyme, aberrant migration of primordial germ cells, or abnormal differentiation or metaplasia of the embryonic coelomic epithelium.
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Affiliation(s)
| | | | | | - Christophe Rosty
- Envoi Pathology, Brisbane, Queensland, Australia.,University of Queensland, Brisbane, Queensland, Australia.,University of Melbourne, Melbourne, Victoria, Australia
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Practical Applications of Molecular Testing in the Cytologic Diagnosis of Pancreatic Cysts. JOURNAL OF MOLECULAR PATHOLOGY 2021. [DOI: 10.3390/jmp2010002] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/06/2023] Open
Abstract
Mucinous pancreatic cysts are precursor lesions of ductal adenocarcinoma. Discoveries of the molecular alterations detectable in pancreatic cyst fluid (PCF) that help to define a mucinous cyst and its risk for malignancy have led to more routine molecular testing in the preoperative evaluation of these cysts. The differential diagnosis of pancreatic cysts is broad and ranges from non-neoplastic to premalignant to malignant cysts. Not all pancreatic cysts—including mucinous cysts—require surgical intervention, and it is the preoperative evaluation with imaging and PCF analysis that determines patient management. PCF analysis includes biochemical and molecular analysis, both of which are ancillary studies that add significant value to the final cytological diagnosis. While testing PCF for carcinoembryonic antigen (CEA) is a very specific test for a mucinous etiology, many mucinous cysts do not have an elevated CEA. In these cases, detection of a KRAS and/or GNAS mutation is highly specific for a mucinous etiology, with GNAS mutations supporting an intraductal papillary mucinous neoplasm. Late mutations in the progression to malignancy such as those found in TP53, p16/CDKN2A, and/or SMAD4 support a high-risk lesion. This review highlights PCF triage and analysis of pancreatic cysts for optimal cytological diagnosis.
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Höhn P, Soydemir MA, Luu AM, Janot-Matuschek M, Tannapfel A, Uhl W, Belyaev O. It's not all about the size-characteristics and risk factors for malignancy of mucinous cystic neoplasms of the pancreas. ANNALS OF TRANSLATIONAL MEDICINE 2020; 8:1572. [PMID: 33437771 PMCID: PMC7791201 DOI: 10.21037/atm-20-4774] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Indexed: 12/28/2022]
Abstract
Background Mucinous cystic neoplasms (MCN) of the pancreas are rare mucin-producing cystic tumors. As they harbor malignant potential, surgical resection is frequently performed. Current guidelines recommend surgery in asymptomatic patients only for MCN exceeding 4 cm. The aim of this study was to identify radiological and clinical risk factors for malignancy in a single-center cohort of MCN. Methods All resected MCN from a single high-volume center between 2004 and 2019 were retrospectively analyzed. Patient characteristics, preoperative findings, histopathological results, and data on the postoperative course were recorded. Variables associated with malignancy were evaluated using χ2 and Mann-Whitney U test. Receiver operating characteristic (ROC) curves were used to model predictive capabilities of preoperative tumor marker levels. Furthermore uni- and multivariate logistic regression analysis were performed for binary variables. Survival time was plotted as Kaplan-Meier curves and evaluated by log-rank test. Results A total of 63 patients were included. Median age was 62 years; 51 (81.0%) of them were women; median tumor size was 3.5 cm (range, 0.5-18.5); 16 (25.4%) of tumors harbored invasive carcinoma and 13 presented intraepithelial dysplasia (20.6%); 7 (43.8%) invasive carcinomas were smaller than 4 cm. All malignant MCN were radiologically suspected of malignancy (calcifications, mural nodules, or wall thickness) preoperatively. Elevated levels of carbohydrate antigen 19-9 (CA19-9) and carcinoembryonic antigen (CEA) were strongly associated with malignancy (odd's ratio 33.600; 7.000-161.270); P<0.001 and odd's ratio 19.250; 3.370-109.970; P<0.001). Other factors associated with malignancy were preoperative weight loss (P=0.015) and higher age (P=0.048). Tumor size, abdominal or back pain or jaundice showed no significant correlation to malignancy in our cohort. Conclusions Malignant potential of MCN should not be underestimated and a close clinical and radiological follow-up is mandatory in all suspected cases. This is especially important for small lesions. Risk assessment should not rely only on tumor size but consider all clinical, radiological and laboratory findings of each case. Follow-up should be performed by experienced surgeons and radiologists in high volume centers for pancreatic surgery. Surgery should be performed in all cases in which malignancy is suspected.
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Affiliation(s)
- Philipp Höhn
- Department of General and Visceral Surgery, St. Josef-Hospital, Ruhr-University Bochum, Bochum, Germany
| | | | - Andreas Minh Luu
- Department of General and Visceral Surgery, St. Josef-Hospital, Ruhr-University Bochum, Bochum, Germany
| | - Monika Janot-Matuschek
- Department of General and Visceral Surgery, St. Josef-Hospital, Ruhr-University Bochum, Bochum, Germany
| | | | - Waldemar Uhl
- Department of General and Visceral Surgery, St. Josef-Hospital, Ruhr-University Bochum, Bochum, Germany
| | - Orlin Belyaev
- Department of General and Visceral Surgery, St. Josef-Hospital, Ruhr-University Bochum, Bochum, Germany
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Li Y, Zhu Z, Peng L, Jin Z, Sun L, Song B. The pathological features and prognoses of intraductal papillary mucinous neoplasm and mucinous cystic neoplasm after surgical resection: a single institution series. World J Surg Oncol 2020; 18:287. [PMID: 33148260 PMCID: PMC7643344 DOI: 10.1186/s12957-020-02063-8] [Citation(s) in RCA: 8] [Impact Index Per Article: 1.6] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/21/2020] [Accepted: 10/21/2020] [Indexed: 02/07/2023] Open
Abstract
BACKGROUND Intraductal papillary mucinous neoplasms (IPMNs) and mucinous cystic neoplasms (MCNs) represent the tumors with malignant transformation potential. The objective of the study was to verify their pathological characteristics, prognoses, and recurrence factors. METHODS Two hundred eighteen IPMNs and 27 MCNs resected at a single institution were included. The demographic, preoperative, histopathological, and follow-up data of the patients were recorded and analyzed. Overall survival (OS) and disease-free survival (DFS) were defined as the interval from the date of initial surgery to death or the last follow-up (OS) and to diagnosis of recurrence or death at follow-up (DFS). RESULTS Of the 218 IPMN and 27 MCN patients, 93 (42.7%) and 8 (29.6%) cases were malignant, respectively. IPMNs occurred in older patients compared with MCN patients (median 63 years vs 54 years, P < 0.0001), and MCNs occurred exclusively in females (100%). Of the overall study cohort, the pathological specimens presented peripheral invasion in 37 (15.1%) patients and incisal margin invasion was observed in 46 (18.8%) patients. After a median follow-up of 34 months, 37 (14.9%) patients relapsed. The 5-year OS and DFS rates of IPMNs were 97.5% and 80.6%; and the OS and DFS rates of MCNs were 95.7% and 87.0%, respectively. There were four independent risk factors associated with recurrence: pathological diagnoses with malignancy (odds ratio, OR = 3.65), presence of oncocytic type for IPMN (OR = 1.69), peripheral invasion (OR = 12.87), and incisal margin invasion (OR = 1.99). CONCLUSIONS IPMNs and MCNs are indolent tumors with favorable prognoses after surgical resection in terms of their relatively high OS and DFS rate. Patients with malignant pathological-related diagnoses should accept strict tumor surveillance in view of their higher risk of recurrence.
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Affiliation(s)
- Yuqiong Li
- Department of Gastroenterology, Changhai Hospital, Navy Medical University, 168 Changhai Road, Shanghai, 200433, China
| | - Zhongfei Zhu
- Department of Hepatobiliary Pancreatic Surgery, Changhai Hospital, Navy Medical University, 168 Changhai Road, Shanghai, 200433, China
| | - Lisi Peng
- Department of Gastroenterology, Changhai Hospital, Navy Medical University, 168 Changhai Road, Shanghai, 200433, China
| | - Zhendong Jin
- Department of Gastroenterology, Changhai Hospital, Navy Medical University, 168 Changhai Road, Shanghai, 200433, China
| | - Liqi Sun
- Department of Gastroenterology, Changhai Hospital, Navy Medical University, 168 Changhai Road, Shanghai, 200433, China.
| | - Bin Song
- Department of Hepatobiliary Pancreatic Surgery, Changhai Hospital, Navy Medical University, 168 Changhai Road, Shanghai, 200433, China.
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Non-coding RNA biomarkers in pancreatic ductal adenocarcinoma. Semin Cancer Biol 2020; 75:153-168. [PMID: 33049362 DOI: 10.1016/j.semcancer.2020.10.001] [Citation(s) in RCA: 38] [Impact Index Per Article: 7.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/26/2020] [Revised: 09/20/2020] [Accepted: 10/02/2020] [Indexed: 12/13/2022]
Abstract
Pancreatic ductal adenocarcinoma (PDAC) is one of the most lethal malignancies, which is usually diagnosed at an advanced stage. The late disease diagnosis, the limited availability of effective therapeutic interventions and lack of robust diagnostic biomarkers, are some of the primary reasons for the dismal 5-year survival rates (∼8%) in patients with PDAC. The pancreatic cancer develops through accumulation of a series of genomic and epigenomic alterations which lead to the transformation of normal pancreatic epithelium into an invasive carcinoma - a process that can take up to 15-20 years to develop, from the occurrence of first initiating mutational event. These facts highlight a unique window of opportunity for the earlier detection of PDAC, which could allow timely disease interception and improvement in the overall survival outcomes in patients suffering from this fatal malignancy. Non-coding RNAs (ncRNAs) have been recognized to play a central role in PDAC pathogenesis and are emerging as attractive candidates for biomarker development in various cancers, including PDAC. More specifically, the ncRNAs play a pivotal role in PDAC biology as they affect tumor growth, migration, and invasion by regulating cellular processes including cell cycle, apoptosis, and epithelial-mesenchymal transition. In this review, we focus on three types of well-established ncRNAs - microRNAs (miRNAs), long non-coding RNAs (lncRNAs), and circular RNAs (circRNAs) - and discuss their potential as diagnostic, prognostic and predictive biomarkers in PDAC.
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Okuse H, Yamada R, Tanaka K, Horiki N, Takei Y. Mucinous cystic adenoma with inflammatory cell infiltration around the splenic artery mimicking pancreatic cancer: a case report. Clin J Gastroenterol 2020; 13:1360-1366. [PMID: 32902779 PMCID: PMC7671980 DOI: 10.1007/s12328-020-01228-y] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/20/2020] [Accepted: 08/27/2020] [Indexed: 11/05/2022]
Abstract
A 45-year-old woman presented with upper abdominal and back pain. A cystic lesion in the pancreas and inflammation around the splenic artery were detected by computed tomography. Although imaging studies were difficult to exclude malignancy, pathological and cytological findings of a fine-needle aspiration showed no signs of malignancy. The patient was, therefore, followed-up for 3 months, during which time the cyst increased in size and developed a cyst-in-cyst structure. She was diagnosed with mucinous cyst neoplasm and underwent distal pancreatectomy. Histologically, the patient was diagnosed as low-grade mucinous cystic adenoma. Soft tissue shadows around the splenic artery were considered to indicate fibrosis and infiltration of inflammatory cells. After distal pancreatectomy, the patient has been uneventful with symptom resolution. This case highlights the potentially atypical presentation of mucinous cystic neoplasms with inflammatory cell infiltration around the splenic artery.
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Affiliation(s)
- Hiroaki Okuse
- Department of Gastroenterology, Suzuka Kaisei Hospital, Suzuka, Mie, Japan
| | - Reiko Yamada
- Department of Gastroenterology and Hepatology, Mie University Hospital, 2-174 Edobashi, Tsu, Mie, 514-8507, Japan.
| | - Kyosuke Tanaka
- Department of Endoscopy, Mie University Hospital, Tsu, Mie, Japan
| | - Noriyuki Horiki
- Department of Endoscopy, Mie University Hospital, Tsu, Mie, Japan
| | - Yoshiyuki Takei
- Department of Gastroenterology and Hepatology, Mie University Hospital, 2-174 Edobashi, Tsu, Mie, 514-8507, Japan
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Intraductal Pancreatic Mucinous Neoplasms: A Tumor-Biology Based Approach for Risk Stratification. Int J Mol Sci 2020; 21:ijms21176386. [PMID: 32887490 PMCID: PMC7504137 DOI: 10.3390/ijms21176386] [Citation(s) in RCA: 11] [Impact Index Per Article: 2.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/13/2020] [Revised: 08/27/2020] [Accepted: 09/01/2020] [Indexed: 12/19/2022] Open
Abstract
Pancreatic ductal adenocarcinoma is one of the most lethal human cancers. Its precursor lesions include pancreatic intra-epithelial neoplasia, mucinous cystic neoplasm, and intraductal papillary mucinous neoplasm (IPMN). IPMNs usually present as an incidental finding at imaging in 2.6% of the population and, according to the degree of dysplasia, they are classified as low- or high-grade lesions. Since the risk of malignant transformation is not accurately predictable, the management of these lesions is based on morphological and clinical parameters, such as presence of mural nodule, main pancreatic duct dilation, presence of symptoms, or high-grade dysplasia. Although the main genetic alterations associated to IPMNs have been elucidated, they are still not helpful for disease risk stratification. The growing body of genomic and epigenomic studies along with the more recent development of organotypic cultures provide the opportunity to improve our understanding of the malignant transformation process, which will likely deliver biomarkers to help discriminate between low- and high-risk lesions. Recent insights on the topic are herein summarized.
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Advances in the management of pancreatic cystic neoplasms. Curr Probl Surg 2020; 58:100879. [PMID: 34144739 DOI: 10.1016/j.cpsurg.2020.100879] [Citation(s) in RCA: 8] [Impact Index Per Article: 1.6] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/15/2020] [Accepted: 08/09/2020] [Indexed: 12/11/2022]
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