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Kumar NA, DSouza AS, Usman N, Bishnoi AK. Agenesis of Dorsal Pancreas and Solid Pseudopapillary Tumor: Ventral Pancreas Preserving Portal Vein Resection and Reconstruction Using a Peritoneal Graft. Cureus 2023; 15:e40916. [PMID: 37496552 PMCID: PMC10366649 DOI: 10.7759/cureus.40916] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 06/24/2023] [Indexed: 07/28/2023] Open
Abstract
A diabetic lady in her 40s was referred to surgical oncologists with epigastric pain associated with vomiting. Computed Tomography (CT) Abdomen with contrast demonstrated a mass arising from the head of the pancreas with the absence of dorsal pancreas, confirmed on magnetic resonance cholangio-pancreatography (MRCP). A core needle biopsy was done, and the tumor was revealed to be a solid pseudopapillary epithelial neoplasm. She underwent sub-total pancreatectomy preserving the duodenum and ventral pancreas as there was adequate free margin; however due to the tumor abutting the anterior wall of the portal vein, it was resected, and reconstruction was done using a peritoneal graft. The patient made a good recovery without any significant post-operative events.
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Affiliation(s)
- Naveena An Kumar
- Surgical Oncology, Kasturba Medical College, Manipal, Udupi, IND
| | - Arika S DSouza
- Surgical Oncology, Kasturba Medical College, Manipal, Udupi, IND
| | - Nawaz Usman
- Surgical Oncology, Kasturba Medical College, Manipal, Udupi, IND
| | - Arvind K Bishnoi
- Cardiothoracic Surgery, Kasturba Medical College, Manipal, Udupi, IND
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2
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Bhattarai M, Shrestha A, Bhandari S, Buha N, Baral R, Shrivastav S, Lakhey PJ. Pancreatic solid pseudopapillary neoplasm with concomitant left unilateral renal agenesis and bicornuate uterus: a case report. Ann Med Surg (Lond) 2023; 85:1166-1171. [PMID: 37113970 PMCID: PMC10129163 DOI: 10.1097/ms9.0000000000000360] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/16/2022] [Accepted: 02/12/2023] [Indexed: 04/29/2023] Open
Abstract
A solid pseudopapillary neoplasm (SPN) of the pancreas is a rare tumor of the pancreas. Concomitant SPN with urogenital anomalies is a very rare presentation. Case Presentation A 16-year-old female presented with a chief complaint of abdominal pain 30 days back. Solid pseudopapillary neoplasm (SPN) of the pancreas was diagnosed with the aid of ultrasonography and contrast-enhanced computed tomography of the abdomen and pelvis. Incidentally, concomitant left unilateral renal agenesis and bicornuate uterus were also detected in radiological findings. The patient underwent spleen-preserving distal pancreatectomy, and SPN was confirmed with the histopathological report. Discussion Symptomatic SPN patients present with an abdominal mass and pain or very rarely jaundice. Most of the SPNs are benign. Complete surgical excision results in more than 95% cure. SPN with concomitant urogenital anomalies is extremely rare, and their concurrent occurrence can be better attributed to Wnt signaling pathway owing to their similar pathogenic mechanism. Conclusion The solid pseudopapillary tumor has an excellent prognosis if timely resected. Proper evaluation of the patient with imaging is necessary to suspect and diagnose SPN who has urogenital anomalies and vice versa.
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Affiliation(s)
- Madhur Bhattarai
- Institute of Medicine, Tribhuvan University, Maharajgunj Medical Campus
| | - Ayush Shrestha
- Institute of Medicine, Tribhuvan University, Maharajgunj Medical Campus
| | - Suyog Bhandari
- Department of Surgical Gastroenterology
- Corresponding author. Address: Institute of Medicine, Tribhuvan University, Maharajgunj 44600, Nepal. Tel. +977-9841881823. E-mail address: (S. Bhandari)
| | | | - Rituraj Baral
- Department of Pathology, Institute of Medicine, Tribhuvan University, Maharajgunj, Nepal
| | - Shreya Shrivastav
- Department of Pathology, Institute of Medicine, Tribhuvan University, Maharajgunj, Nepal
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Lalchandani A, Maurya A, Rizvi SFM, Yadav A. Agenesis of the Dorsal Pancreas: A Rare Cause of Diabetes and Recurrent Upper Abdominal Pain. Cureus 2023; 15:e34689. [PMID: 36909095 PMCID: PMC9994769 DOI: 10.7759/cureus.34689] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 02/05/2023] [Indexed: 02/08/2023] Open
Abstract
Agenesis of the dorsal pancreas is a rare congenital disorder with only a handful of cases described in the literature. It presents a diagnostic dilemma. Cross-sectional imaging is the cornerstone for diagnosis. It could have a syndromic association with polysplenia and cardiac anomalies. Pancreas divisum and chronic pancreatitis may present with similar symptoms and must be ruled out. We present a case of a 55-year-old male with recurrent non-specific abdominal pain and diabetes mellitus. He was managed with insulin and painkillers for symptomatic relief. We also reviewed approximately 68 cases described in the literature to date.
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Affiliation(s)
- Ankit Lalchandani
- General Surgery, All India Institute of Medical Sciences Bhopal, Bhopal, IND
| | - Ajeet Maurya
- General Surgery, All India Institute of Medical Sciences Bhopal, Bhopal, IND
| | | | - Amit Yadav
- General Surgery, All India Institute of Medical Sciences Bhopal, Bhopal, IND
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4
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Shyr BS, Wang SE, Chen SC, Shyr YM, Shyr BU. Pancreatic head sparing surgery for solid pseudopapillary tumor in patients with agenesis of the dorsal pancreas. J Chin Med Assoc 2022; 85:981-986. [PMID: 35801950 DOI: 10.1097/jcma.0000000000000771] [Citation(s) in RCA: 6] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/26/2022] Open
Abstract
BACKGROUND This study aimed to clarify the feasibility and justification of pancreatic head sparing (PHS) enucleation for patients with agenesis of the dorsal pancreas (ADP) associated with a solid pseudopapillary tumor (SPT). METHODS Data of the SPT patients with and without ADP, including clinical presentations, surgical options, and surgical and survival outcomes, were recruited for comparison. RESULTS A total of 31 patients with SPTs were included, three of whom displayed ADP and underwent PHS enucleation. Surgical complications were comparable between the groups. Overall, the 5- and 10-year disease-free survival rates were 100% and 90%, respectively. The 20- and 25-year overall survival rates were 100% and 66.7%, respectively. Only one patient (3.2%) developed tumor recurrence 7.3 years after pancreatectomy for an SPT with lymph node involvement, and the patient survived 24.5 years after the initial operation. No tumor recurrence occurred in any patient with ADP after PHS enucleation. CONCLUSION PHS enucleation seems to be feasible and justifiable for SPT patients with ADP in terms of surgical and survival outcomes, and this approach could be recommended to avoid pancreatic insufficiency.
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Affiliation(s)
- Bor-Shiuan Shyr
- Division of General Surgery, Department of Surgery, Taipei Veterans General Hospital, Taipei, Taiwan, ROC
- Department of Surgery, National Yang Ming Chiao Tung University, Taipei, Taiwan, ROC
| | - Shin-E Wang
- Division of General Surgery, Department of Surgery, Taipei Veterans General Hospital, Taipei, Taiwan, ROC
- Department of Surgery, National Yang Ming Chiao Tung University, Taipei, Taiwan, ROC
| | - Shih-Chin Chen
- Division of General Surgery, Department of Surgery, Taipei Veterans General Hospital, Taipei, Taiwan, ROC
- Department of Surgery, National Yang Ming Chiao Tung University, Taipei, Taiwan, ROC
| | - Yi-Ming Shyr
- Division of General Surgery, Department of Surgery, Taipei Veterans General Hospital, Taipei, Taiwan, ROC
- Department of Surgery, National Yang Ming Chiao Tung University, Taipei, Taiwan, ROC
| | - Bor-Uei Shyr
- Division of General Surgery, Department of Surgery, Taipei Veterans General Hospital, Taipei, Taiwan, ROC
- Department of Surgery, National Yang Ming Chiao Tung University, Taipei, Taiwan, ROC
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5
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Complete agenesis of dorsal pancreas with pancreatic cyst: A case report. North Clin Istanb 2021; 8:307-309. [PMID: 34222814 PMCID: PMC8240227 DOI: 10.14744/nci.2020.24444] [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] [Subscribe] [Scholar Register] [Received: 04/23/2020] [Accepted: 04/29/2020] [Indexed: 01/10/2023] Open
Abstract
Agenesis of the dorsal pancreas (ADP) is extremely rare disease with no specific symptoms and there is no clear pathogenesis. Approximately half of the affected individuals develop diabetes resulting from reduced islet cell mass secondary to lack of endocrine structures. In this case, we aimed to present a 17-year-old female patient with ADP accompanied by a pancreatic cyst.
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6
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Three cases of pancreatic pseudocysts associated with dorsal pancreatic agenesis. Radiol Case Rep 2018; 14:79-82. [PMID: 30377453 PMCID: PMC6202790 DOI: 10.1016/j.radcr.2018.09.023] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/10/2018] [Revised: 09/16/2018] [Accepted: 09/28/2018] [Indexed: 02/06/2023] Open
Abstract
Agenesis of the dorsal pancreas (ADP) is an extremely rare congenital anomaly. Human pancreas is formed by ventral and dorsal endodermal buds of the foregut endoderm. The dorsal bud forms the upper part of the head, neck, body, and tail of the pancreas and the ventral bud generates most of the head and uncinate process. ADP is derived from the embryologic failure of the dorsal pancreatic bud to form the pancreatic body and tail. ADP can be related to some diseases and conditions such as pancreatitis, hypoglycemia, and rarely pancreatic tumors. The association between cystic lesions with ADP has previously been reported. Three cases of cystic lesions of the pancreas with ADP were diagnosed clinically based on the imaging features and without any past history of pancreatitis. However, the pathologic diagnosis of resected lesions confirmed pseudocysts without pathologic evidence of tumor. We report 3 cases of pancreatic pseudocysts associated with ADP
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Erotokritou A, Gerharz CD, Sagir A. Agenesis of dorsal pancreas associated with pancreatic neuroendocrine tumor: a case report and review of the literature. J Med Case Rep 2018; 12:185. [PMID: 29958547 PMCID: PMC6026343 DOI: 10.1186/s13256-018-1733-9] [Citation(s) in RCA: 7] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/24/2018] [Accepted: 05/31/2018] [Indexed: 11/10/2022] Open
Abstract
Background Agenesis of the dorsal pancreas is very rare. Less than 70 cases have been reported to date. Some of these cases had an association with a tumor. The literature of agenesis of the dorsal pancreas and agenesis of the dorsal pancreas-associated pancreatic neoplasia is limited. Here we report the second case of a pancreatic neuroendocrine tumor in a setting of agenesis of the dorsal pancreas. Case presentation A 71-year-old man, originally from North Africa, with a history of insulin-dependent diabetes mellitus, presented with a 2-month history of nonspecific abdominal symptoms. Contrast-enhanced computed tomography demonstrated an almost 3 cm round, quite well-defined and homogeneous tumor formation in the area between the neck and absent body and tail of his pancreas. The mass was confirmed by endoscopic ultrasound. Our patient underwent computed tomography-guided biopsy of the mass which provided proof of a neuroendocrine tumor. He underwent pancreas resection because of the presence of a neuroendocrine tumor. Seven months later his glycated hemoglobin increased from 6.9 to 8.7%. Conclusions Diagnosis of agenesis of the dorsal pancreas is based on imaging techniques like computed tomography, magnetic resonance cholangiopancreatography, or endoscopic ultrasound. Endoscopic ultrasound-guided fine-needle aspiration can be helpful for the histological diagnosis of the tumor. The hypothesis of the association between pancreatic neoplasia and agenesis of the dorsal pancreas leads us to the suggestion that every patient with diagnosed agenesis of the dorsal pancreas should be observed with a focus on the early detection of potential malignancy.
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Affiliation(s)
- A Erotokritou
- Department of Gastroenterology, Academic Teaching Hospital Bethesda Duisburg, Heerstr, 219 47053, Duisburg, Germany
| | - C D Gerharz
- Department of Pathology, Academic Teaching Hospital Bethesda Duisburg, Heerstr. 219, 47053, Duisburg, Germany
| | - A Sagir
- Department of Gastroenterology, Academic Teaching Hospital Bethesda Duisburg, Heerstr, 219 47053, Duisburg, Germany.
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Saikaly E, El Asmar A, Abi Fadel F, Aoun M, El Rassi Z. Agenesis of the dorsal pancreas associated with mucinous adenocarcinoma and cystic teratoma: a case report and literature review. Clin Case Rep 2017; 5:175-181. [PMID: 28174646 PMCID: PMC5290498 DOI: 10.1002/ccr3.797] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/06/2016] [Revised: 11/18/2016] [Accepted: 12/08/2016] [Indexed: 11/07/2022] Open
Abstract
Dorsal agenesis of the pancreas is a rare congenital anomaly. Fifty‐eight cases were reported from 1913 till 2015, nine of which were associated with tumors. We present the 10th case, the first to be associated with pancreatic mucinous adenocarcinoma and cystic teratoma, successfully managed by Whipple procedure and total pancreatectomy.
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Affiliation(s)
- Elias Saikaly
- Faculty of MedicineSaint Georges Hospital University Medical CenterUniversity of BalamandBeirutLebanon
| | - Antoine El Asmar
- Faculty of MedicineSaint Georges Hospital University Medical CenterUniversity of BalamandBeirutLebanon
| | - Fahim Abi Fadel
- Faculty of MedicineSaint Georges Hospital University Medical CenterUniversity of BalamandBeirutLebanon
| | - Mona Aoun
- Faculty of MedicineSaint Georges Hospital University Medical CenterUniversity of BalamandBeirutLebanon
| | - Ziad El Rassi
- General and Digestive SurgeryOncologic SurgeryFaculty of MedicineSaint Georges Hospital University Medical CenterUniversity of BalamandBeirutLebanon
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9
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Pancreatic Neuroendocrine Tumor in the Setting of Dorsal Agenesis of the Pancreas. Case Rep Gastrointest Med 2016; 2016:3801962. [PMID: 27738535 PMCID: PMC5050349 DOI: 10.1155/2016/3801962] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/04/2016] [Revised: 08/29/2016] [Accepted: 08/30/2016] [Indexed: 12/18/2022] Open
Abstract
Dorsal agenesis of the pancreas (DAP) is an uncommon embryological abnormality where there is absence of the distal pancreas. DAP is mostly asymptomatic, but common presenting symptoms include diabetes mellitus, abdominal pain, pancreatitis, enlarged pancreatic head, and, in a few cases, polysplenia. MRCP and ERCP are the gold standard imaging techniques to demonstrate the absence of the dorsal pancreatic duct. The literature on the association of pancreatic neoplasia and DAP is limited. We present the case of a pancreatic neuroendocrine tumor in a patient with dorsal agenesis of the pancreas, with a review of the related literature.
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10
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Paiella S, De Pastena M, Esposito A, Salvia R, Morigi C, Bassi C. Selective agenesis of pancreatic isthmus parenchyma with preservation of main pancreatic duct continuity, a very rare entity: Case report. Int J Surg Case Rep 2014; 6C:169-71. [PMID: 25544484 PMCID: PMC4347963 DOI: 10.1016/j.ijscr.2014.09.023] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/16/2014] [Accepted: 09/18/2014] [Indexed: 12/03/2022] Open
Abstract
Pancreatic congenital anomalies are not infrequent. A rare selective agenesis of pancreatic parenchyma is presented. Neoplastic lesions should always been considered in case of pancreatic congenital anomalies. Context Agenesis of the dorsal pancreas is a rare anomaly, mostly associated with other medical conditions. It may be complicated with pancreatic neoplasms. Case report We report the case of a 51-year-old male with selective agenesis of pancreatic isthmus with preservation of main pancreatic duct and branch-duct intraductal papillary mucinous neoplasm (BD-IPMN) with suspicious features of the pancreas. Conclusion This is probably the first report of isolated agenesis of pancreatic isthmus with conservation of main pancreatic duct.
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Affiliation(s)
- Salvatore Paiella
- University of Verona, Department of General Surgery B, Istituto del Pancreas, Verona, Italy.
| | - Matteo De Pastena
- University of Verona, Department of General Surgery B, Istituto del Pancreas, Verona, Italy
| | - Alessandro Esposito
- University of Verona, Department of General Surgery B, Istituto del Pancreas, Verona, Italy
| | - Roberto Salvia
- University of Verona, Department of General Surgery B, Istituto del Pancreas, Verona, Italy
| | - Consuelo Morigi
- University of Verona, Department of General Surgery B, Istituto del Pancreas, Verona, Italy
| | - Claudio Bassi
- University of Verona, Department of General Surgery B, Istituto del Pancreas, Verona, Italy
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Gagnière J, Dupré A, Ines DD, Tixier L, Pezet D, Buc E. Giant mucinous cystic adenoma with pancreatic atrophy mimicking dorsal agenesis of the pancreas. World J Gastrointest Surg 2014; 6:42-46. [PMID: 24672649 PMCID: PMC3964414 DOI: 10.4240/wjgs.v6.i3.42] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/01/2013] [Revised: 01/15/2014] [Accepted: 02/18/2014] [Indexed: 02/06/2023] Open
Abstract
Mucinous cystic adenoma (MCA) of the pancreas is a rare benign cystic tumor with ovarian-like stroma and lack of communication with the pancreatic ductal system. The ovarian tissue is incorporated from the left gonad within the dorsal pancreas during embryogenesis. Consequently, congenital dorsal agenesis of the pancreas (DAP) cannot be associated with MCA. We report the case of a giant MCA associated with atrophy of the dorsal pancreas mimicking complete DAP. Pancreato-magnetic resonance imaging failed to identify the dorsal pancreas but the absence of diabetes mellitus and compression of the splenic vein with major tributaries rectified the diagnosis of secondary atrophy of the distal pancreas. Unusual proximal location of the cyst in the pancreas may have induced chronic obstruction of both the dorsal pancreatic duct and the splenic vein, with secondary atrophy of the distal pancreas.
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12
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Guan ZW, Sun L, Wang YQ, Xu BX. Solid pseudopapillary tumor of the pancreas and concomitant urogenital malformations in a young woman. Diagn Pathol 2013; 8:35. [PMID: 23445554 PMCID: PMC3606420 DOI: 10.1186/1746-1596-8-35] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/20/2012] [Accepted: 02/20/2013] [Indexed: 02/06/2023] Open
Abstract
Abstract Solid pseudopapillary tumor (SPT) of the pancreas is a rare pancreatic tumor with low malignant potential. It occurs characteristically more often in young women. SPT associated with extra- and pancreatic anomalies are occasionally reported. Here we report a case of pancreatic SPT with concomitant urogenital malformations including solitary kidney and uterus didelphys in a 25-year-old woman. The patient underwent central pancreatectomy, and SPT was confirmed with pathological results. Recurrence or metastasis was not found after 14 months of follow-up. Virtual Slides The virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/4264758678755142
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Affiliation(s)
- Zhi-Wei Guan
- Department of Nuclear Medicine, Chinese PLA General Hospital, Ruxing road 28, Beijing 100853, China
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13
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14
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Abstract
Morphogenesis of the pancreas is a complex process; nevertheless, congenital anomalies are rare. At embryogenesis, the pancreas develops from the endoderm-lined dorsal and ventral buds of the duodenum. The ventral bud gives rise to the lower head and uncinate process of the pancreas; whereas, the dorsal bud gives rise to the upper head, isthmus, body, and tail of the pancreas. Rarely, developmental failure of the dorsal pancreatic bud at embryogenesis results in the agenesis of the dorsal pancreas--neck, body, and tail. Even rarer is the association of pancreatic tumors with agenesis of the dorsal pancreas. In addition to citing our case, we provide a comprehensive review on agenesis of the dorsal pancreas and its association with pancreatic tumors.
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15
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Schnedl WJ, Piswanger-Soelkner C, Wallner SJ, Reittner P, Krause R, Lipp RW, Hohmeier HE. Agenesis of the dorsal pancreas and associated diseases. Dig Dis Sci 2009; 54:481-7. [PMID: 18618254 DOI: 10.1007/s10620-008-0370-3] [Citation(s) in RCA: 56] [Impact Index Per Article: 3.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/17/2008] [Accepted: 06/03/2008] [Indexed: 12/13/2022]
Abstract
BACKGROUND Agenesis of the dorsal pancreas is a very rare congenital pancreatic malformation and is associated with some other diseases. METHODS A PubMed search revealed 53 cases of agenesis of the dorsal pancreas. RESULTS In 28 patients with this congenital malformation hyperglycemia was demonstrated, 27 had abdominal pain, 16 had pancreatitis, 14 had an enlarged or prominent pancreatic head visible on computed tomography, and in a few cases, polysplenia, which may occur with various congenital anomalies of visceral organs, was described. CONCLUSIONS Difficulties involved in obtaining a firm diagnosis have led to a variety of terms being used to describe this congenital disease. Diagnosis of agenesis of the dorsal pancreas is inconclusive without demonstration of the absence of the dorsal pancreatic duct. Here we describe the embryological development of the pancreas, the so-far known cases of agenesis of the dorsal pancreas with associated medical problems, and the diagnostic measures to find the right conclusions.
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Affiliation(s)
- Wolfgang J Schnedl
- Department of Internal Medicine, Medical University, Auenbruggerplatz 15, A-8036, Graz, Austria.
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16
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17
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Peng CH, Chen DF, Zhou GW, Yang WP, Tao ZY, Lei RQ, Zhange SD, Li HW. The solid-pseudopapillary tumor of pancreas: the clinical characteristics and surgical treatment. J Surg Res 2006; 131:276-82. [PMID: 16457845 DOI: 10.1016/j.jss.2005.11.585] [Citation(s) in RCA: 36] [Impact Index Per Article: 1.9] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/23/2005] [Revised: 11/02/2005] [Accepted: 11/28/2005] [Indexed: 12/13/2022]
Abstract
BACKGROUND AND OBJECTIVE Since being named and reclassified by WHO in 1996, solid-pseudopapillary tumor (SPT) of pancreas has been recognized as a special entitative disease that is different from pancreatic cancer and should be recognized and treated more accurately in the surgical process. The clinic characteristics and surgical strategy on 25 cases of SPT of pancreas from the authors' center are discussed. METHODS The clinical pathology and the surgical methods of 25 SPTs were retrospectively studied. The analyses were performed by the statistical software package SAS6.12. RESULTS No tumor recurrences were found in all patients. There was significant difference between operative types in radical resection and the tumor position of the pancreas (P = 0.0011). The judgment on the tumor's boundary could directly affect the adoptable operative types (P = 0.0099). CONCLUSIONS As a uniquely entitative disease, SPT is a kind of uncommon neoplasm with low-grade malignancy with a strong rate of occurrence in women. Surgical resection is most favorable in the treatment of SPT, which has excellent prognosis. The course of SPT, the possible malignant cells by the frozen section biopsy, and the tumor's boundary are important for operators to decide an operative scheme. SPT that has infiltrated contiguous vessels, organs, even with local liver metastasis should not be regarded as operative contraindication. The choice of the local tumor resection, the part of pancreas resection or radical resection depends on the judgment of the tumor's boundary, whereas operative types in radical resection depend on the tumor position of the pancreas.
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Affiliation(s)
- Cheng-hong Peng
- Department of Surgery and the Center of the Organ Transplantation, Shanghai Institute of Digestive Surgery, Ruijin Hospital, Shanghai Second Medical University, Shanghai, China.
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18
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Mirković D, Karamarković A. [Solid-pseudopapillary tumors of the pancreas]. VOJNOSANIT PREGL 2005; 62:763-7. [PMID: 16305105 DOI: 10.2298/vsp0510763m] [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: 11/27/2022] Open
Abstract
BACKGROUND Solid-pseudopapillary tumors of the pancreas (SPT) is a very rare lesion. We presented the clinical and pathological characteristics of this tumor and reviewed the literature. CASE REPORT We analysed the treatment of the two female patients, aged 21 and 37 years, with this tumor. The first female patient had the tumor withont involvement of the surrounding organs. The second female patient had the tumor with a high grade of malignancy which invaded blood vessels. We performed in both patients splenohemipancreatectomy, and then we reported the pathological characteristics of the tumors. We didn't find recidive in the observed postoperative follow-up of 4, and 6 years, respectively. CONCLUSION SPT is a rare tumor of the pancreas that is diagnosed primarily in young women. The prognosis after surgical treatment was excellent. The histopathological analysis is the main procedure in establishing diagnosis, regardless the most recent diagnostic procedures.
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Affiliation(s)
- Darko Mirković
- Vojnomedicinska akademija, Klinika za opstu i vaskularnu hirurgiju, Beograd, Srbija i Crna Gora
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Kasem A, Ali Z, Ellul J. Papillary cystic and solid tumour of the pancreas: report of a case and literature review. World J Surg Oncol 2005; 3:62. [PMID: 16188022 PMCID: PMC1261538 DOI: 10.1186/1477-7819-3-62] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/23/2005] [Accepted: 09/27/2005] [Indexed: 11/10/2022] Open
Abstract
Background The papillary cystic and solid tumour of the pancreas (PCSTP) is a rare primary neoplasm of unknown pathogenesis typically found in young women. PCSTP is a low-grade malignant tumour, which is often asymptomatic but it may present with abdominal pain. Case presentation A 38 year old female patient who presented with one day history of epigastric pain was diagnosed as PCSTP. The patient was successfully treated with distal pancreatectomy. Conclusion It is important to differentiate this tumour from other pancreatic tumours because, unlike malignant pancreatic tumours, this neoplasm does not usually metastasise and is amenable to cure after complete surgical resection. However, the cell origin and the aetiology of this tumour are not clear and further studies are warranted in its pathogenesis.
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Affiliation(s)
- Abdul Kasem
- Department of General Surgery, Princess Royal University Hospital,Farnborough Common, Orpington, BR6 8DN UK
| | - Zainab Ali
- Department of Pathology, Princess Royal University Hospital,Farnborough Common, Orpington, BR6 8DN UK
| | - Joseph Ellul
- Department of General Surgery, Princess Royal University Hospital,Farnborough Common, Orpington, BR6 8DN UK
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Ulusan S, Bal N, Kizilkilic O, Bolat F, Yildirim S, Yildirim T, Niron EA. Case report: solid-pseudopapillary tumour of the pancreas associated with dorsal agenesis. Br J Radiol 2005; 78:441-3. [PMID: 15845940 DOI: 10.1259/bjr/91312352] [Citation(s) in RCA: 22] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/14/2022] Open
Abstract
Solid-pseudopapillary tumour of the pancreas is a rare benign or low-grade malignant epithelial tumour; its association with pancreatic dorsal agenesis has been reported only once before. We present the radiological and histological findings of a case of pancreatic solid-pseudopapillary tumour associated with total pancreatic dorsal agenesis. A 49-year-old woman presented with abdominal pain radiating to the back for several months. Radiological findings showed absence of the dorsal pancreas and an 8 cm x 6 cm diameter tumour arising from the head of the pancreas. She underwent successful complete resection of the tumour. Histopathology revealed a diagnosis of solid-pseudopapillary tumour.
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Affiliation(s)
- S Ulusan
- Department of Radiology, Baskent University Adana Teaching and Medical Research Centre, Adana, Turkey
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Karamarković AR, Micev M, Culafić D, Popović N, Djukić V. Solid-cystic-pseudopapillarytumor of the pancreas - case report. SRP ARK CELOK LEK 2004; 132:431-4. [PMID: 15938224 DOI: 10.2298/sarh0412431k] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/07/2023] Open
Abstract
Solid-cystic-pseudopapillary tumor (SCPT) of the pancreas, described by Frantz (1959), is a very rare clinical pathologic entity with relatively low grade malignant potential. The tumor is more frequent in the body and tail of the pancreas. The majority of patients are young females. About 60% of patients are asymptomatic. Complications such as rupture, bleeding or secondary infections are rare. Metastases of the tumor and local recurrence after surgical treatment are also rare. Prognosis is excellent after complete surgical removal. It is difficult to make a preoperative diagnosis of pancreatic SCPT. The exact diagnosis is based on histological findings. Differential diagnosis should consider pancreatoblastoma, non neoplastic cysts, pancreatic pseudo-cysts and hydatid cyst. This is a case report of 39-year old woman who was admitted to our institution with abdominal discomfort and palpable abdominal mass in the upper abdomen. US and CT scan revealed round neoformation of 60 mm in diameter located in the body of the pancreas. Imaging features were not specific enough to allow for precise diagnosis. Curative R0 left spleno-hemipancreatectomy was performed. Histology of the resected specimen revealed solid and cystic-pseudopapillary tumor of the pancreas. The patient was discharged on postoperative day 7 in a good condition. The patient is well 48 months after the operation, with no impairment of pancreatic endocrine or exocrine function.
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