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Alshawwa K, Njoum Y, Abdul-Hafez HA, Bannoura S, Abukeshek T, Ashhab H, Abu-Zaydeh O. Pancreatic solid pseudopapillary neoplasm, rare presentation in pediatric age group: two case reports. Front Oncol 2025; 15:1528793. [PMID: 40144210 PMCID: PMC11936897 DOI: 10.3389/fonc.2025.1528793] [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: 11/15/2024] [Accepted: 02/17/2025] [Indexed: 03/28/2025] Open
Abstract
Solid pseudopapillary neoplasm (SPN) is a rare tumor that primarily affects young females. It is typically found in the pancreas and is often asymptomatic until it grows to a large size. SPN is more frequently located in the body or tail of the pancreas in adults, whereas in children, it is more commonly found in the head of the pancreas. In this report we present two female patients in the pediatric age group who were diagnosed with pancreatic SPN, each presenting with nonspecific symptoms, one with recurrent epigastric pain and nausea, the other with carpopedal spasms. Imaging showed large, cystic-solid pancreatic masses in both. Each case underwent a Whipple procedure (pancreaticoduodenectomy) with R0 resection. Pathology confirmed SPNs without lymphovascular or perineural invasion, and all surgical margins were clear. This series underscores SPN's varied presentations and favorable surgical outcomes in young adolescents. SPN is a rare neoplasm with low malignant potential that can present as a large abdominal mass. Although surgical resection is the treatment of choice, the optimal surgical approach remains controversial. Early detection and timely management are essential for a favorable outcome. Clinicians should consider SPN in the differential diagnosis of young females presenting with epigastric or pancreatic masses. This report highlights the importance of early detection and timely management of SPN.
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Affiliation(s)
- Khaled Alshawwa
- Department of General Surgery, Al-Makassed Charitable Society Hospital, Jerusalem, Palestine
| | - Yumna Njoum
- Faculty of Medicine, Al-Quds University, Jerusalem, Palestine
| | - Hamza A. Abdul-Hafez
- Department of Medicine, Faculty of Medicine and Health Sciences, An-Najah National University, Nablus, Palestine
| | - Sami Bannoura
- Department of Pathology, Al-Makassed Charitable Society Hospital, Jerusalem, Palestine
| | - Tawfiq Abukeshek
- Department of Radiology, Al-Makassed Charitable Society Hospital, Jerusalem, Palestine
| | - Hazem Ashhab
- Department of Gastroenterology, Al-Quds University Medical School, Jerusalem, Palestine
| | - Omar Abu-Zaydeh
- Department of General Surgery, Al-Makassed Charitable Society Hospital, Jerusalem, Palestine
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Fleming AM, Gehle DB, Freitas JP, Hendrick LE, Yakoub D, Abdelhafeez H, Nezakatgoo N, Deneve JL, Langham MR, Glazer ES, Shibata D, Merchant NB, Dickson PV, Murphy AJ. CTNNB1 exon 3 mutations in metastatic solid pseudopapillary neoplasm of the pancreas. J Surg Oncol 2024; 130:1051-1061. [PMID: 39155692 DOI: 10.1002/jso.27808] [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: 07/19/2024] [Accepted: 07/29/2024] [Indexed: 08/20/2024]
Abstract
BACKGROUND AND OBJECTIVES Solid pseudopapillary neoplasm (SPN) of the pancreas demonstrates an indolent disease course; however, some patients present with a "malignant" phenotype, including distant metastases resistant to chemotherapy. This analysis identifies molecular drivers of metastatic SPN using the world's largest clinicogenomics database. METHODS The American Association for Cancer Research Project Genomics Evidence Neoplasia Information Exchange was queried for primary and metastatic SPN samples. Sample-level genomic alterations were compared. A pan-pancreatic cancer analysis assessed relevant mutations among all metastatic pancreatic malignancies. RESULTS Among 28 SPN samples identified (n = 17 primary, n = 11 metastatic), the most commonly mutated gene was CTNNB1, (24/28 samples; 85.7%). Most mutations were missense (21/24; 87.5%) or in-frame deletions (3/24; 12.5%). The most common CTNNB1 mutations in primary SPN were exon 3 S37F/C missense mutations (6/16 profiled patients, 37.5%), contrasting exon 3 D32N/Y/H missense mutations in metastatic samples (6/11 profiled patients, 54.5%). Metastatic SPN had higher rates of CTNNB1 mutations than metastases from pancreatic ductal adenocarcinoma (72.7% vs. 1.1%; q < 0.0001), pancreatic neuroendocrine tumor (72.7% vs. 2.5%; q < 0.0001), and pancreatic acinar cell carcinoma (72.7% vs. 11.5%; q = 0.0254). CONCLUSIONS Missense mutations along exon 3 of CTNNB1 predominate metastatic SPN, differentiating these patients from those with metastases from analogous pancreatic malignancies.
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Affiliation(s)
- Andrew M Fleming
- Department of Surgery, University of Tennessee Health Science Center, Memphis, Tennessee, USA
- Department of Surgery, St. Jude Children's Research Hospital, Memphis, Tennessee, USA
| | - Daniel B Gehle
- Department of Surgery, University of Tennessee Health Science Center, Memphis, Tennessee, USA
- Department of Surgery, St. Jude Children's Research Hospital, Memphis, Tennessee, USA
| | - Julia Pedo Freitas
- Department of Surgery, University of Tennessee Health Science Center, Memphis, Tennessee, USA
| | - Leah E Hendrick
- Division of Gastrointestinal Oncology, H. Lee Moffitt Cancer Center, Tampa, Florida, USA
| | - Danny Yakoub
- Department of Surgery, Augusta University Medical Center, Augusta, Georgia, USA
| | - Hafeez Abdelhafeez
- Department of Surgery, University of Tennessee Health Science Center, Memphis, Tennessee, USA
- Department of Surgery, St. Jude Children's Research Hospital, Memphis, Tennessee, USA
| | - Nosratollah Nezakatgoo
- Department of Surgery, University of Tennessee Health Science Center, Memphis, Tennessee, USA
- Department of Surgery, St. Jude Children's Research Hospital, Memphis, Tennessee, USA
| | - Jeremiah L Deneve
- Department of Surgery, University of North Carolina, Chapel Hill, North Carolina, USA
| | - Max R Langham
- Department of Surgery, University of Tennessee Health Science Center, Memphis, Tennessee, USA
- Department of Surgery, St. Jude Children's Research Hospital, Memphis, Tennessee, USA
| | - Evan S Glazer
- Department of Surgery, University of Tennessee Health Science Center, Memphis, Tennessee, USA
- Department of Surgery, St. Jude Children's Research Hospital, Memphis, Tennessee, USA
| | - David Shibata
- Department of Surgery, University of Tennessee Health Science Center, Memphis, Tennessee, USA
- Department of Surgery, St. Jude Children's Research Hospital, Memphis, Tennessee, USA
| | - Nipun B Merchant
- Department of Surgery, University of Miami Health System, Miami, Florida, USA
| | - Paxton V Dickson
- Department of Surgery, University of Tennessee Health Science Center, Memphis, Tennessee, USA
- Department of Surgery, St. Jude Children's Research Hospital, Memphis, Tennessee, USA
| | - Andrew J Murphy
- Department of Surgery, University of Tennessee Health Science Center, Memphis, Tennessee, USA
- Department of Surgery, St. Jude Children's Research Hospital, Memphis, Tennessee, USA
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Harada A, Kurobe M, Miyaguni K, Sugihara T, Kaji S, Kanamori D, Uchida G, Baba Y, Hiramatsu T, Ohashi S. Clinical Insights Into Pediatric Solid Pseudopapillary Neoplasms of the Pancreas. Cureus 2024; 16:e70655. [PMID: 39493013 PMCID: PMC11527602 DOI: 10.7759/cureus.70655] [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: 10/01/2024] [Indexed: 11/05/2024] Open
Abstract
BACKGROUND Solid pseudopapillary neoplasms (SPNs) of the pancreas are rare tumors with low malignant potential that usually occur in young girls and women. This study investigated the treatment experiences and outcomes after surgery for pediatric SPNs in our institutions as pediatric case series of solid SPNs are few, and long-term follow-up is also limited. METHODS We retrospectively reviewed the outcomes of nine patients diagnosed with SPNs who underwent surgery in our three hospitals (The Jikei University Hospital, The Jikei University Kashiwa Hospital, and Kawaguchi Municipal Medical Center) between 2001 and 2023. RESULTS All nine patients who underwent surgery were girls. Their ages ranged from 8 to 15 years (median: 10 years). The location of the tumor was at the pancreatic head, body, and tail in five, one, and three patients, respectively. Enucleation, pancreaticoduodenectomy, and laparoscopic distal pancreatectomy (LDP) were performed in four, two, and three patients, respectively. Regarding postoperative complications, a pancreatic fistula was detected in six patients, with three and three patients having grades A and B fistulas, respectively; two patients required percutaneous drainage, and one patient required endoscopic ultrasonography (EUS)-guided transgastric drainage. The follow-up period ranged from six to 261 months (median: 97 months). At the final follow-up, all nine patients were alive without recurrence. CONCLUSION SPNs of the pancreas are incidentally diagnosed by diagnostic workups following trauma in children more frequently compared to adults. Additionally, the tumor resection by minimally invasive approaches, such as enucleation, or laparoscopic procedures results in a good prognosis in some cases.
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Affiliation(s)
- Atsushi Harada
- Pediatric Surgery, Kawaguchi Municipal Medical Center, Kawaguchi, JPN
| | - Masashi Kurobe
- Pediatric Surgery, The Jikei University School of Medicine, Tokyo, JPN
| | - Kazuaki Miyaguni
- Pediatric Surgery, The Jikei University School of Medicine, Tokyo, JPN
| | - Tetsuro Sugihara
- Pediatric Surgery, The Jikei University School of Medicine, Tokyo, JPN
| | - Sayuri Kaji
- Pediatric Surgery, The Jikei University School of Medicine, Tokyo, JPN
| | - Daisuke Kanamori
- Pediatric Surgery, The Jikei University School of Medicine, Tokyo, JPN
| | - Goki Uchida
- Pediatric Surgery, The Jikei University School of Medicine, Tokyo, JPN
| | - Yuji Baba
- Pediatric Surgery, The Jikei University School of Medicine, Tokyo, JPN
| | | | - Shinsuke Ohashi
- Pediatric Surgery, The Jikei University School of Medicine, Tokyo, JPN
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Ligurgo O, Averous G, Addeo P. Acute pancreatitis and large cystic pancreatic tumor in an adolescent. J Gastrointest Surg 2024; 28:776-778. [PMID: 38704211 DOI: 10.1016/j.gassur.2024.01.032] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/26/2024] [Accepted: 01/27/2024] [Indexed: 05/06/2024]
Affiliation(s)
- Oronzo Ligurgo
- Hepato-Pancreato-Biliary Surgery and Liver Transplantation, Pôle des Pathologies Digestives, et Hépatiques, Hôpital de Hautepierre-Hôpitaux Universitaires de Strasbourg, Université de Strasbourg, Strasbourg, France
| | - Gerlinde Averous
- Hepato-Pancreato-Biliary Surgery and Liver Transplantation, Pôle des Pathologies Digestives, et Hépatiques, Hôpital de Hautepierre-Hôpitaux Universitaires de Strasbourg, Université de Strasbourg, Strasbourg, France
| | - Pietro Addeo
- Hepato-Pancreato-Biliary Surgery and Liver Transplantation, Pôle des Pathologies Digestives, et Hépatiques, Hôpital de Hautepierre-Hôpitaux Universitaires de Strasbourg, Université de Strasbourg, Strasbourg, France.
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Xu YC, Fu DL, Yang F. Unraveling the enigma: A comprehensive review of solid pseudopapillary tumor of the pancreas. World J Gastrointest Oncol 2024; 16:614-629. [PMID: 38577449 PMCID: PMC10989376 DOI: 10.4251/wjgo.v16.i3.614] [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: 11/09/2023] [Revised: 12/28/2023] [Accepted: 01/16/2024] [Indexed: 03/12/2024] Open
Abstract
Solid pseudopapillary tumor of the pancreas (SPTP) is a rare neoplasm predominantly observed in young females. Pathologically, CTNNB1 mutations, β-catenin nuclear accumulation, and subsequent Wnt-signaling pathway activation are the leading molecular features. Accurate preoperative diagnosis often relies on imaging techniques and endoscopic biopsies. Surgical resection remains the mainstay treatment. Risk models, such as the Fudan Prognostic Index, show promise as predictive tools for assessing the prognosis of SPTP. Establishing three types of metachronous liver metastasis can be beneficial in tailoring individualized treatment and follow-up strategies. Despite advancements, challenges persist in understanding its etiology, establishing standardized treatments for unresectable or metastatic diseases, and developing a widely recognized grading system. This comprehensive review aims to elucidate the enigma by consolidating current knowledge on the epidemiology, clinical presentation, pathology, molecular characteristics, diagnostic methods, treatment options, and prognostic factors.
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Affiliation(s)
- Ye-Cheng Xu
- Department of Pancreatic Surgery, Pancreatic Disease Institute, Huashan Hospital, Fudan University, Shanghai 200040, China
| | - De-Liang Fu
- Department of Pancreatic Surgery, Pancreatic Disease Institute, Huashan Hospital, Fudan University, Shanghai 200040, China
| | - Feng Yang
- Department of Pancreatic Surgery, Pancreatic Disease Institute, Huashan Hospital, Fudan University, Shanghai 200040, China
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Radulovic MK, Brecelj J, Gruden A, Flezar MS. Role of endoscopic ultrasound-guided fine needle aspiration biopsies in diagnosing pancreatic neoplasms in the paediatric population: experience from a tertiary center and review of the literature. Radiol Oncol 2024; 58:78-86. [PMID: 38378032 PMCID: PMC10878772 DOI: 10.2478/raon-2024-0008] [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: 07/14/2023] [Accepted: 10/07/2023] [Indexed: 02/22/2024] Open
Abstract
BACKGROUND Endoscopic ultrasound-guided fine needle aspiration biopsy (EUS FNAB) is a well established diagnostic method in adult patients, but is rarely used in the paediatric population. The Clinical Department of Gastroenterology at the University Clinical Centre Ljubljana and the Department of Cytopathology at the Institute of Pathology, Faculty of Medicine, University of Ljubljana, Slovenia, have been closely collaborating on EUS FNAB since the introduction in 2010. The aim of the study was to review the cases of EUS FNAB of pancreatic neoplasms in children. PATIENTS AND METHODS In the digital archive of the Institute of Pathology (IP), Faculty of Medicine (FM), University of Ljubljana (UL), we found 6 cases of EUS FNAB in children, 3 had EUS FNAB of the pancreas, 2 of whom had a cytopathologic diagnosis of a tumour. In the first case, the lesion was ultrasonographically solid, and the cell sample contained branching papillary structures surrounded by aggregates of small cells with nuclear grooves. In the second case, the lesion was ultrasonographically cystic, and predominantly necrosis was seen, with only single preserved cells. Positive nuclear reaction for β-catenin was found in both cases by immunohistochemical staining. RESULTS In both cases, the cytopathological diagnosis of solid pseudopapillary neoplasm of the pancreas was made, the cases represent the totality of paediatric cases of pancreatic neoplasms from the Children's Hospital Ljubljana since 2010. There were no adverse events during and after EUS FNAB. A histopathological examination of the tumour resection specimens confirmed the cytopathological diagnosis. CONCLUSIONS Our experience indicates that EUS FNAB is a safe and effective method for diagnosing pancreatic neoplasms in the pediatric population, as supported by the findings in the literature.
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Affiliation(s)
- Maja Kebe Radulovic
- Institute of Pathology, Faculty of Medicine, University of Ljubljana, Ljubljana, Slovenia
| | - Jernej Brecelj
- Department of Gastroenterology, Hepatology and Nutrition, University Children’s Hospital Ljubljana, and Department of Pediatrics, Faculty of Medicine, University of Ljubljana, Ljubljana, Slovenia
| | - Andrej Gruden
- University Medical Centre Ljubljana, Service of Internal Medicine, Department of Gastroenterology, Ljubljana, Slovenia
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Fleming AM, Mansfield SA, Jancelewicz T, Gosain A, Eubanks JW, Davidoff AM, Langham MR, Murphy AJ. Hepatic Metastasectomy in Pediatric Patients: An Observational Study. J Pediatr Surg 2024; 59:247-253. [PMID: 37980196 DOI: 10.1016/j.jpedsurg.2023.10.036] [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: 10/07/2023] [Accepted: 10/11/2023] [Indexed: 11/20/2023]
Abstract
BACKGROUND The role of hepatectomy for metastatic disease in children is controversial. Rationales include potential cure, obtaining a diagnosis, and guiding chemotherapy decisions. This study examines the safety and utility of hepatic metastasectomy for children at a single institution. METHODS After IRB approval (#22-1258), medical records were reviewed from 1995 to 2022 for children undergoing hepatic metastasectomy. En-bloc hepatectomies during primary tumor resection were excluded. RESULTS Hepatic metastasectomy was performed in 16 patients for a variety of histologies. Median patient age was 12.2 years [IQR 6.9-22.6], and 13/16 patients were female (81 %). Number of hepatic metastases ranged from 1 to 23 and involved between 1 and 8 Couinaud segments. Anatomic resections included 4 hemihepatectomies and 1 sectionectomy. All other resections were nonanatomic. 3/6 resections for germ cell tumor (GCT) revealed only mature teratoma, driving adjuvant therapy decisions. When indicated, median time to adjuvant chemotherapy was 19 days [IQR 11-22]. No patients had Clavien-Dindo Class III or higher perioperative morbidity. Three patients (1 GCT, 1 adrenocortical carcinoma (ACC), and 1 gastric neuroendocrine tumor (GNET) experienced hepatic relapse. The patients with relapsed GCT and GNET are alive with disease at 17 and 135 months, respectively. The patient with ACC died of disease progression and liver failure. One patient with Wilms tumor experienced extrahepatic, retroperitoneal recurrence and died. With a median follow-up of 38 months, 10-year disease-specific and disease-free survival were 77 % and 61 %, respectively. CONCLUSIONS Hepatic metastasectomy can be accomplished safely in children, may guide adjuvant therapy decisions, and is associated with long-term survival in selected patients. LEVEL OF EVIDENCE Level IV. TYPE OF STUDY Treatment Study, Case series with no comparison group.
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Affiliation(s)
- Andrew M Fleming
- St. Jude Children's Research Hospital, Department of Surgery, Memphis, TN, USA; The University of Tennessee Health Science Center, Department of Surgery, Memphis, TN, USA
| | - Sara A Mansfield
- St. Jude Children's Research Hospital, Department of Surgery, Memphis, TN, USA; The University of Tennessee Health Science Center, Department of Surgery, Memphis, TN, USA
| | - Tim Jancelewicz
- St. Jude Children's Research Hospital, Department of Surgery, Memphis, TN, USA; The University of Tennessee Health Science Center, Department of Surgery, Memphis, TN, USA
| | - Ankush Gosain
- St. Jude Children's Research Hospital, Department of Surgery, Memphis, TN, USA; The University of Tennessee Health Science Center, Department of Surgery, Memphis, TN, USA
| | - James W Eubanks
- St. Jude Children's Research Hospital, Department of Surgery, Memphis, TN, USA; The University of Tennessee Health Science Center, Department of Surgery, Memphis, TN, USA
| | - Andrew M Davidoff
- St. Jude Children's Research Hospital, Department of Surgery, Memphis, TN, USA; The University of Tennessee Health Science Center, Department of Surgery, Memphis, TN, USA
| | - Max R Langham
- St. Jude Children's Research Hospital, Department of Surgery, Memphis, TN, USA; The University of Tennessee Health Science Center, Department of Surgery, Memphis, TN, USA
| | - Andrew J Murphy
- St. Jude Children's Research Hospital, Department of Surgery, Memphis, TN, USA; The University of Tennessee Health Science Center, Department of Surgery, Memphis, TN, USA.
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ALGhabra Y, Hamdi M, Alhomsi M, Alusef A, Qatleesh S, Ousta MA. Solid pseudopapillary neoplasm of the pancreas: A case report with a brief literature review. Int J Surg Case Rep 2024; 115:109234. [PMID: 38245948 PMCID: PMC10831808 DOI: 10.1016/j.ijscr.2024.109234] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/19/2023] [Revised: 01/03/2024] [Accepted: 01/05/2024] [Indexed: 01/23/2024] Open
Abstract
INTRODUCTION Solid pseudopapillary neoplasm (SPN) of the pancreas, representing only 1 % of pancreatic cancers, was identified by Virginia Frantz in 1959. Predominantly affecting young females, it often remains asymptomatic, posing diagnostic challenges due to slow growth. This paper emphasizes SPN's rarity and associated diagnostic complexities. CASE PRESENTATION In a specific case, a 17-year-old female with post-traumatic right flank pain underwent an enhanced CT scan, revealing a well-defined, hypodense mass in the pancreatic head. With normal laboratory results, a diagnostic laparotomy exposed a sizable solid cystic mass. A Whipple procedure unveiled a predominantly cystic mass enveloped by a well-developed capsule. DISCUSSION SPN appears as a distinct mixed solid and cystic lesion on imaging, necessitating confirmation through core biopsy. Surgical resection, the primary treatment, ensures a positive overall prognosis, despite rare recurrence and metastases. Microscopic examination reveals pseudopapillae, and immunohistochemistry aids diagnosis with positive staining for estrogen receptor, progesterone receptor, CD10, and CD99. CONCLUSION SPN, a rare pancreatic neoplasm predominantly affecting young females, may present with abdominal pain or palpable mass despite its usual asymptomatic nature. Diagnosis involves imaging and biopsy confirmation, with surgical resection as the curative treatment. While prognosis is generally favorable, comprehensive understanding and improved management require further research for this uncommon pancreatic neoplasm.
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Affiliation(s)
- Yasser ALGhabra
- Al-Mouwasat University Hospital, Faculty of Medicine, Damascus University, Damascus, Syria
| | - Mohammad Hamdi
- Al-Mouwasat University Hospital, Faculty of Medicine, Damascus University, Damascus, Syria; M.D, Faculty of Medicine, Aleppo University, Aleppo, Syria.
| | - Malath Alhomsi
- Al-Mouwasat University Hospital, Faculty of Medicine, Damascus University, Damascus, Syria
| | - Ahmad Alusef
- Al-Mouwasat University Hospital, Faculty of Medicine, Damascus University, Damascus, Syria; M.D, Faculty of Medicine, Aleppo University, Aleppo, Syria
| | - Safaa Qatleesh
- Al-Mouwasat University Hospital, Faculty of Medicine, Damascus University, Damascus, Syria
| | - Mhd Ali Ousta
- Al-Mouwasat University Hospital, Faculty of Medicine, Damascus University, Damascus, Syria
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Fleming AM, Dickson PV, Murphy AJ. ASO Author Reflections: "Malignant" Solid Pseudopapillary Neoplasm of the Pancreas? Ann Surg Oncol 2024; 31:546-547. [PMID: 37857981 DOI: 10.1245/s10434-023-14434-y] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/20/2023] [Accepted: 09/26/2023] [Indexed: 10/21/2023]
Affiliation(s)
- Andrew M Fleming
- Department of Surgery, The University of Tennessee Health Science Center, Memphis, TN, USA.
- Department of Surgery, St. Jude Children's Research Hospital, Memphis, TN, USA.
| | - Paxton V Dickson
- Department of Surgery, The University of Tennessee Health Science Center, Memphis, TN, USA
- Department of Surgery, St. Jude Children's Research Hospital, Memphis, TN, USA
| | - Andrew J Murphy
- Department of Surgery, The University of Tennessee Health Science Center, Memphis, TN, USA
- Department of Surgery, St. Jude Children's Research Hospital, Memphis, TN, USA
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10
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Fleming AM, Hendrick LE, Yakoub D, Abdelhafeez H, Deneve JL, Langham MR, Glazer ES, Davidoff AM, Merchant NB, Dickson PV, Murphy AJ. Malignant Solid Pseudopapillary Neoplasm of the Pancreas: An Orthogonal Analysis. Ann Surg Oncol 2024; 31:475-487. [PMID: 37768414 DOI: 10.1245/s10434-023-14343-0] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/18/2023] [Accepted: 09/06/2023] [Indexed: 09/29/2023]
Abstract
BACKGROUND Pancreatic solid pseudopapillary neoplasms (SPN) are generally indolent; however, some patients present with "malignant" SPN. An orthogonal analysis of multiple datasets was performed to investigate the utility of complete surgical resection (CSR) for malignant SPN. METHODS A systematic review was performed for cases of malignant SPN, defined as T4, N1, and/or M1. Malignant SPN was analyzed within the National Cancer Database (NCDB) and compared with T1-3N0M0 SPN. Predictors of malignant SPN were assessed, and treatments were analyzed by using survival analysis. RESULTS The systematic review yielded 164 cases of malignant SPN. Of 31 children, only one died due to malignant SPN. Among adults, CSR was associated with improved disease-specific survival (DSS) (P = 0.0002). Chemotherapy did not improve malignant SPN DSS, whether resected (P = 0.8485) or not (P = 0.2219). Of 692 adults with SPN within the NCDB, 93 (13.4%) had malignant SPN. Pancreatic head location (odds ratio [OR] 2.174; 95% confidence interval [CI] 1.136-4.166; P = 0.0186) and tumor size (OR 1.154; 95% CI 1.079-1.235; P < 0.0001) associated with the malignant phenotype. Malignant SPN predicted decreased overall survival (OS) compared with T1-3N0M0 disease (P < 0.0001). Resected malignant SPN demonstrated improved OS (P < 0.0001), including resected stage IV malignant SPN (P = 0.0003). Chemotherapy did not improve OS for malignant SPN, whether resected (P = 0.8633) or not (P = 0.5734). Within a multivariable model, resection was associated with decreased hazard of death (hazard ratio 0.090; 95% CI 0.030-0.261; P < 0.0001). CONCLUSIONS Approximately 13% of patients with SPN present with a malignant phenotype. Pediatric cases may be less aggressive. Resection may improve survival for malignant SPN, which does not appear chemosensitive.
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Affiliation(s)
- Andrew M Fleming
- Department of Surgery, The University of Tennessee Health Science Center, Memphis, TN, USA.
- Department of Surgery, St. Jude Children's Research Hospital, Memphis, TN, USA.
| | - Leah E Hendrick
- Department of Surgery, The University of Tennessee Health Science Center, Memphis, TN, USA
| | - Danny Yakoub
- Department of Surgery, Augusta University Medical Center, Augusta, GA, USA
| | - Hafeez Abdelhafeez
- Department of Surgery, The University of Tennessee Health Science Center, Memphis, TN, USA
- Department of Surgery, St. Jude Children's Research Hospital, Memphis, TN, USA
| | - Jeremiah L Deneve
- Department of Surgery, University of North Carolina, Chapel Hill, NC, USA
| | - Max R Langham
- Department of Surgery, The University of Tennessee Health Science Center, Memphis, TN, USA
- Department of Surgery, St. Jude Children's Research Hospital, Memphis, TN, USA
| | - Evan S Glazer
- Department of Surgery, The University of Tennessee Health Science Center, Memphis, TN, USA
| | - Andrew M Davidoff
- Department of Surgery, The University of Tennessee Health Science Center, Memphis, TN, USA
- Department of Surgery, St. Jude Children's Research Hospital, Memphis, TN, USA
| | - Nipun B Merchant
- Department of Surgery, University of Miami Health System, Miami, FL, USA
| | - Paxton V Dickson
- Department of Surgery, The University of Tennessee Health Science Center, Memphis, TN, USA
| | - Andrew J Murphy
- Department of Surgery, The University of Tennessee Health Science Center, Memphis, TN, USA
- Department of Surgery, St. Jude Children's Research Hospital, Memphis, TN, USA
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11
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Baskaran S, Lodha M, Kumar RR, Elhence P. Spontaneous rupture of solid pseudopapillary epithelial neoplasm of pancreas with intraperitoneal haemorrhage. BMJ Case Rep 2023; 16:e257398. [PMID: 38081741 PMCID: PMC10728932 DOI: 10.1136/bcr-2023-257398] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/18/2023] Open
Affiliation(s)
- Suruthi Baskaran
- General Surgery, All India Institute of Medical Sciences, Jodhpur, Rajasthan, India
| | - Mahendra Lodha
- General Surgery, All India Institute of Medical Sciences, Jodhpur, Rajasthan, India
| | - Ravi Ranjan Kumar
- General Surgery, All India Institute of Medical Sciences, Jodhpur, Rajasthan, India
| | - Poonam Elhence
- Pathology, All India Institute of Medical Sciences, Jodhpur, Rajasthan, India
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Jentzsch C, Fuchs J, Agaimy A, Vokuhl C, Escherich G, Blattmann C, Warmann SW, Schmidt A, Schäfer J, Brecht IB, Schneider DT, Abele M. Solid pseudopapillary neoplasms of the pancreas in childhood and adolescence-an analysis of the German Registry for Rare Pediatric Tumors (STEP). Eur J Pediatr 2023; 182:5341-5352. [PMID: 37733117 PMCID: PMC10746605 DOI: 10.1007/s00431-023-05203-w] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/01/2023] [Revised: 09/09/2023] [Accepted: 09/12/2023] [Indexed: 09/22/2023]
Abstract
Solid pseudopapillary neoplasms (SPNs) are the most common entity among pediatric pancreatic tumors. Still, these are rare tumors with an annual incidence of 0.1-0.2/1,000,000, and little is known about their optimal treatment. This analysis aimed to increase knowledge about the occurrence and treatment strategies of SPN in childhood. Data regarding diagnostics, treatment, and outcome of children aged 0-18 years with SPN recorded in the German Registry for Rare Pediatric Tumors (STEP) were analyzed. Thirty-eight patients were identified with a median age of 14.5 years at diagnosis (range: 8-18) and a female preponderance (81.6%). The most frequent location of the tumor was the pancreatic tail. In histopathological and immunohistochemical examination, pseudopapillary, solid, and cystic lesions as well as expression of beta-catenin, progesterone receptors, and cyclin D1 were the most common findings. All patients underwent surgical resection. Most patients underwent open resection, predominantly tail resection for tumors in the tail region and pylorus-preserving pancreaticoduodenectomy for tumors in the head region. The main postoperative sequela was exogenous pancreatic insufficiency (23.7%), especially with SPN in the pancreatic head. No recurrence occurred during follow-up, although two patients underwent resection with microscopic residue. CONCLUSION SPN of the pancreas in childhood are low-grade malignancies with usually favorable treatment outcomes. However, therapy can lead to relevant long-term sequelae. To prevent recurrence, complete surgical resection is recommended, sparing as much healthy pancreatic tissue as possible. Interdisciplinary collaboration between specialists is essential to optimize treatment. Molecular genetic analysis of these tumors could improve understanding of their genesis. WHAT IS KNOWN • Solid pseudopapillary neoplasms (SPNs) of the pancreas are very rare tumors in childhood. • Little is known about tumorigenesis, and there are no specific guidelines for treatment and follow-up in pediatric patients. WHAT IS NEW • Characteristics, treatment, and outcome were comprehensively assessed in a large cohort of pediatric patients with SPN. • We propose recommendations for diagnosis, treatment, and follow-up of children with SPN, based on our analysis and considering published experience.
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Affiliation(s)
- Christian Jentzsch
- Pediatric Hematology/Oncology, Department of Pediatrics, University Hospital Tuebingen, Tuebingen, Germany
| | - Jörg Fuchs
- Pediatric Surgery and Pediatric Urology, Department of Pediatrics, University Hospital Tuebingen, Tuebingen, Germany
| | - Abbas Agaimy
- Institute of Pathology, University Hospital Erlangen, Erlangen, Germany
| | - Christian Vokuhl
- Section of Pediatric Pathology, Department of Pathology, University Hospital Bonn, Bonn, Germany
| | - Gabriele Escherich
- Department of Pediatric Hematology and Oncology, University Medical Center Hamburg-Eppendorf, Hamburg, Germany
| | - Claudia Blattmann
- Department of Pediatric Oncology, Hematology and Immunology, Olgahospital, Klinikum Stuttgart, Stuttgart, Germany
| | - Steven W Warmann
- Pediatric Surgery and Pediatric Urology, Department of Pediatrics, University Hospital Tuebingen, Tuebingen, Germany
| | - Andreas Schmidt
- Pediatric Surgery and Pediatric Urology, Department of Pediatrics, University Hospital Tuebingen, Tuebingen, Germany
| | - Jürgen Schäfer
- Section of Pediatric Radiology, Department of Diagnostic and Interventional Radiology, University Hospital Tuebingen, Tuebingen, Germany
| | - Ines B Brecht
- Pediatric Hematology/Oncology, Department of Pediatrics, University Hospital Tuebingen, Tuebingen, Germany
| | - Dominik T Schneider
- Clinic of Pediatrics, Klinikum Dortmund, University Witten/Herdecke, Dortmund, Germany
| | - Michael Abele
- Pediatric Hematology/Oncology, Department of Pediatrics, University Hospital Tuebingen, Tuebingen, Germany.
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13
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Luo W, Chen H, Wang J, Qiu J, Zhang T. Comprehensive study of clinical features, prognostic factors, and survival in patients with pancreatic solid pseudopapillary neoplasms based on the 2019 WHO classification. J Cancer Res Clin Oncol 2023; 149:12393-12404. [PMID: 37438539 DOI: 10.1007/s00432-023-04982-x] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/23/2023] [Accepted: 06/10/2023] [Indexed: 07/14/2023]
Abstract
BACKGROUND Pancreatic solid pseudopapillary neoplasms (pSPNs) are a rare tumor type with a limited understanding of their clinical characteristics and survival outcomes. We aimed to investigate potential prognostic factors among the existing clinical features in patients diagnosed with pSPN. METHODS For this study, we utilized data from the Surveillance Epidemiology and End Results (SEER) database, specifically selecting patients with a histology type of pSPN from the years 2000 to 2019. Subsequently, we conducted both univariate and multivariate Cox regression analyses in a systematic manner to identify potential prognostic factors associated with overall survival (OS) and cancer-specific survival (CSS) in the selected group of patients. To assess the disparity in OS and CSS among different clinical features and treatments, Kaplan-Meier curves were generated. Furthermore, utilizing the results obtained from the multivariate analysis, we developed a nomogram predictive model to effectively forecast the prognosis of patients diagnosed with pSPN. Calibration plots were presented to demonstrate the predictive accuracy and reliability of the nomogram predictive model. RESULTS The study comprised a total of 433 participants, with 85.7% of the patients diagnosed with pSPN being female and the remaining 14.3% being male. The Kaplan-Meier curves indicated that patients with pSPN who underwent primary tumor resection (PTR) and those who were younger than 70 years old had significantly improved OS and CSS compared to those who did not undergo PTR or were aged 70 years or older, respectively (P < 0.001). Male patients diagnosed with pSPN exhibited poor OS compared to female pSPN patients (P = 0.015). The multivariate Cox regression analysis indicated that age (OS: HR = 1.055, 95% CI = 1.027-1.084, P < 0.001. CSS: HR = 1.054, 95% CI = 1.019-1.091, P = 0.002) and PTR (OS: HR = 6.074, 95% CI = 1.922-19.194, P = 0.002. CSS: HR = 4.912, 95% CI = 1.188-20.312, P = 0.028) were independent prognostic factors for both OS and CSS. Moreover, tumor size (≥ 5 vs < 5 cm CSS: HR = 4.788, 95% CI = 1.012-22.661, P = 0.048) was an independent prognostic factor for CSS. The independent prognostic factors identified through the multivariate Cox regression analysis were utilized to construct a nomogram model for predicting both OS and CSS in patients with pSPN. The accuracy of the nomogram model was visually testified by the calibration plot with acceptable predictive performance. CONCLUSION Although the majority of patients diagnosed with pSPN are females, it was observed that male patients tend to have poorer OS compared to their female counterparts. The independent prognostic factors identified in the study were age and PTR, which were associated with both OS and CSS. Tumor size was an independent prognostic factor for CSS. Patients who underwent PTR exhibited improved OS and CSS outcomes. The developed nomogram and corresponding reference table provided promising prognostic predictions for pSPN outcoms, serving as a valuable resource for clinicians and patients alike.
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Affiliation(s)
- Wenhao Luo
- Department of General Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, 100730, China
| | - Hao Chen
- Department of General Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, 100730, China
| | - Jun Wang
- Department of General Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, 100730, China
| | - Jiangdong Qiu
- Department of General Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, 100730, China
| | - Taiping Zhang
- Department of General Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, 100730, China.
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Stefanova N, Kalinov T, Kolev N, Kalchev E. Frantz Tumor: A Case Report of Solid Pseudopapillary Tumor of Pancreas. Cureus 2023; 15:e41698. [PMID: 37575792 PMCID: PMC10413994 DOI: 10.7759/cureus.41698] [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: 07/10/2023] [Indexed: 08/15/2023] Open
Abstract
The solid pseudopapillary tumor (SPT) is a rare pancreatic lesion that usually affects young and middle-aged patients and has a female predominance and low malignant potential. The exact histogenesis of this tumor is still unclear. We present the case of a 60-year-old female patient with occasional abdominal pain. Positron emission tomography/computed tomography (PET/CT) and magnetic resonance imaging (MRI) revealed a tumor mass in the pancreatic tail. Distal pancreatectomy and splenectomy were performed. The result from the pathology report was solid pseudopapillary neoplasm (SPN). The patient underwent four cycles of adjuvant chemotherapy with gemcitabine, which she tolerated well without complaints. A control computed tomography (CT) scan and PET/CT of the abdomen (five months after the operation) showed a cystic lesion suspicious for local recurrence in the pancreatic tail during the follow-up period. The patient underwent a second surgery operation. Subsequent histological examination showed chronic indurative pancreatitis, areas with steatonecrosis, lipogranulomas, and fibrosis without evidence of relapse. SPT is a rare pancreatic tumor that most commonly affects young women. Although the tumor has locally aggressive characteristics, the prognosis is excellent after surgical excision. Our case emphasizes that this tumor can occur not only in young women but also in older patients. Chronic granulomatous inflammation and indurative pancreatitis can sometimes mimic a relapse on CT and PET/CT image tests.
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Affiliation(s)
- Nadezhda Stefanova
- Department of General and Clinical Pathology, Forensic Medicine and Deontology, Medical University of Varna, "St. Marina" University Hospital, Varna, BGR
| | - Turgay Kalinov
- Department of General Surgery, Medical University of Varna, "St. Marina" University Hospital, Varna, BGR
| | - Nikola Kolev
- Department of General Surgery, Medical University of Varna, "St. Marina" University Hospital, Varna, BGR
| | - Emilian Kalchev
- Department of Diagnostic Imaging and Radiotherapy, Medical University of Varna, "St. Marina" University Hospital, Varna, BGR
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15
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Vieira RMF, Souza Junior ASD, Kerche LE. CYSTIC PANCREATIC LESIONS: IMAGING VERSUS ANATOMOPATHOLOGICAL FINDINGS-HOW TO IMPROVE DIAGNOSTIC ACCURACY? ARQUIVOS BRASILEIROS DE CIRURGIA DIGESTIVA : ABCD = BRAZILIAN ARCHIVES OF DIGESTIVE SURGERY 2023; 36:e1735. [PMID: 37255104 DOI: 10.1590/0102-672020230017e1735] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Subscribe] [Scholar Register] [Received: 08/14/2022] [Accepted: 01/15/2023] [Indexed: 06/01/2023]
Abstract
BACKGROUND Pancreatic cystic lesions are a group of pancreatic neoplasms with different behavior and risk of malignancy. Imaging diagnosis and differentiation of these lesions remain a challenge. AIMS The aim of this study was to evaluate the agreement between computed tomography and/or magnetic resonance imaging and post-operative pathologic diagnoses of Pancreatic cystic lesions in a University Hospital of São Paulo State. METHODS A total of 39 patients with surgically diagnosed Pancreatic cystic lesions were enrolled, as a study cohort from 2009 to 2019. Preoperative radiological and final pathological diagnosis was correlated to measure computed tomography and/or magnetic resonance imaging diagnostic. Pancreatic adenocarcinoma, choledochal pancreatic cyst, mucinous cystadenoma, serous cystadenoma, intraductal papillary mucinous neoplasms, and pancreatic pseudocyst were classified as neoplastic cysts. RESULTS It was noted that 27 patients (69.23%) had preoperative computed tomography and magnetic resonance imaging, 11 patients (28.20%) had preoperative computed tomography only, and 1 patient had preoperative magnetic resonance imaging only. The values for diagnoses made only with computed tomography (p=0.47) and from the combination of computed tomography+magnetic resonance imaging (p=0.50) did also point to moderate agreement with the anatomopathological findings. The values pointed to a fair agreement for the diagnosis of mucinous cystadenoma (p=0.3), moderate agreement for intraductal papillary mucinous neoplasms (p= 0.41), good agreement for serous cystadenoma (p=0.79), and excellent agreement for choledochal pancreatic cyst (p=1), pancreatic pseudocyst (p=0.84), and Frantz tumor (p=1) (p<0.05). CONCLUSIONS The findings of computed tomography and/or magnetic resonance imaging have an equivalent diagnostic agreement with an anatomopathological diagnosis for differentiating benign from malignant Pancreatic cystic lesions and in suggesting a specific diagnosis. There is no statistical difference between the use of computed tomography alone and computed tomography+magnetic resonance imaging in the improvement of diagnostic accuracy.
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Affiliation(s)
- Rafael Mello Fontolan Vieira
- Cancer Regional Hospital, Hepatobiliopancreatic Oncological Digestive Surgery - Presidente Prudente (SP), Brazil
| | | | - Leandra Ernst Kerche
- Faculty of Medicine of Oeste Paulista, Functional Sciences Department - Presidente Prudente (SP), Brazil
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16
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Singh S, Zhao Q, Sachs TE, Hartshorn K. Reporting a Case of Solid Pseudopapillary Neoplasm of the Pancreas in a 44-Year-Old Woman with Parallel Analysis of Literature. Case Rep Oncol Med 2023; 2023:1768926. [PMID: 37153719 PMCID: PMC10162882 DOI: 10.1155/2023/1768926] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/01/2022] [Revised: 03/31/2023] [Accepted: 04/10/2023] [Indexed: 05/10/2023] Open
Abstract
We present a distinctive case of solid pseudopapillary neoplasm as seen in a 44-year-old woman who presented with an abdominal mass but unremarkable labs with no elevation in any of the tumor markers. Her symptomatology ranged from typical symptoms suggestive of malignancy such as weight loss, lethargy, and anorexia to complaints like abdominal pain and jaundice. Prior to presenting at our center, she was given no hope or much in terms of treatment options. She was found to have a substantial mass over the body and tail of pancreas with characteristic and typical gross as well as histological features. Subsequently, she underwent a successful surgery and has found herself in remission since.
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Affiliation(s)
- Sargun Singh
- Division of Gastroenterology and Liver Disease, Case Western Reserve University School of Medicine, Cleveland, OH, USA
| | - Qing Zhao
- Department of Pathology, Boston University School of Medicine, Boston, MA, USA
| | - Teviah E. Sachs
- Section of Surgical Oncology, Department of Surgery, Boston University School of Medicine, Boston, MA, USA
| | - Kevan Hartshorn
- Section of Hematology Oncology, Department of Medicine, Boston University School of Medicine, Boston, MA, USA
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17
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Abstract
Early detection of high-risk pancreatic cystic lesions enables potentially curative surgical resection, and early detection of lesions without worrisome features may lead to appropriate surveillance. Regrettably, differentiating premalignant and malignant cysts from nonmalignant ones remains challenging. However, emerging additional diagnostic tools, including the needle biopsy with microforceps and needle-based confocal laser endomicroscopy, are of exciting potential along with cyst fluid analysis".
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Affiliation(s)
- Sahin Coban
- Department of Gastroenterology, Mount Auburn Hospital, 330 Mt Auburn St, Cambridge, MA 02138, USA.
| | - Omer Basar
- Department of Gastroenterology, The University of Missouri, Columbia, MO 65211, USA
| | - William R Brugge
- Department of Gastroenterology, Harvard Medical School, Mount Auburn Hospital, 330 Mt Auburn St, Cambridge, MA 02138, USA
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18
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Park S, Park MG, Roh YH, Kim DK. Solid Pseudopapillary Neoplasm of the Pancreas with Lymph Node Metastasis in a Young Male Patient. THE KOREAN JOURNAL OF GASTROENTEROLOGY 2022; 79:77-82. [PMID: 35232923 DOI: 10.4166/kjg.2021.133] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Subscribe] [Scholar Register] [Received: 09/30/2021] [Revised: 11/12/2021] [Accepted: 11/12/2021] [Indexed: 11/03/2022]
Abstract
Solid pseudopapillary pancreatic neoplasms are rare. The male-to-female ratio is 1:9, and metastasis occurs only in a few cases. A 39-year-old male with a solid pseudopapillary neoplasm (SPN) with lymph node metastasis underwent ultrasonography, CT, and MRI, which revealed a mass (8 cm) in the pancreatic head. Fluorodeoxyglucose (FDG)-PET showed a hypermetabolic lymph node in the root area of the superior mesenteric artery (SMA). The patient underwent pylorus-preserving pancreaticoduodenectomy, which confirmed a peripancreatic lymph node metastasis. The lymph node of the SMA root area remained because of the encasing of the superior mesenteric artery. After 14 months of follow-up (with no adjuvant therapy initiated), the residual metastatic lymph nodes showed no change and no recurrence. In conclusion, surgery of the primary tumor for patients with SPN is recommended, even in cases with metastatic lymph nodes remaining.
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Affiliation(s)
- Seyeon Park
- Department of Internal Medicine, Dong-A University College of Medicine, Busan, Korea
| | - Min Gyoung Park
- Department of Pathology, Dong-A University College of Medicine, Busan, Korea
| | - Young Hoon Roh
- Department of Surgery, Dong-A University College of Medicine, Busan, Korea
| | - Dong Kyun Kim
- Department of Internal Medicine, Dong-A University College of Medicine, Busan, Korea
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19
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Madadi-Sanjani O, Zöller C, Beilken A, Pfister ED, Sander B, Ure B, Dingemann J. [Solid Pseudopapillary Neoplasm of the Pancreas in Children and Adolescents - Tailored Diagnostic and Operative Concepts in Four Patients]. KLINISCHE PADIATRIE 2022; 234:228-235. [PMID: 35189655 DOI: 10.1055/a-1733-2229] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 10/19/2022]
Abstract
BACKGROUND Pancreatic tumors in children and adolescents are rare entities that can stay asymptomatic for long periods. They often become apparent as incidental findings or due to clinical symptoms, like abdominal pain. Solid pseudopapillary neoplasms of the pancreas (SPN) are rare representatives of this group, being low-grade malignant processes and requiring radical surgical treatment. We present four cases of SPN with different diagnostic and therapeutic approaches. METHODS A retrospective analysis of four cases of SPN treated between 2015 and 2020 was performed. RESULTS Four female patients (11-17 years) were found to have SPN during diagnostic procedures. Three of them were located in the pancreatic head. Histological confirmation was obtained with endosonographic-, CT-guided and open biopsy, respectively. R0 resection was achieved by a pylorus preserving, partial duodenopancreatectomy according to Traverso-Longmire. In one patient the tumor was located in the pancreatic tail with tumor adherence to the splenic vessels. A CT guided biopsy confirmed an SPN. A distal pancreatectomy and splenectomy was performed. Follow-up (6 months - 6 years) revealed no evidence of tumor recurrence, metastasis, or pancreoprive diabetes. CONCLUSION For the treatment plan preoperative histological confirmation of SPN is necessary. Based on the tailored diagnostic and operative concepts, treatment at a center with a specialized pediatric surgery, pediatric oncology, pediatric gastroenterology, pediatric radiology and pathology is mandatory.
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Affiliation(s)
- Omid Madadi-Sanjani
- Department of Paediatric Surgery, Hannover Medical School, Hannover, Germany
| | - Christoph Zöller
- Department of Paediatric Surgery, University Hospital Muenster, Muenster, Germany
| | - Andreas Beilken
- Department of Pediatric Hematology and Oncology, Hannover Medical School, Hannover, Germany
| | - Eva-Doreen Pfister
- Department of Paediatric Gastroenterology and Hepatology, Hannover Medical School, Hannover, Germany
| | - Björn Sander
- Institute of Pathology, Hannover Medical School, Hannover, Germany
| | - Benno Ure
- Department of Paediatric Surgery, Hannover Medical School, Hannover, Germany
| | - Jens Dingemann
- Department of Paediatric Surgery, Hannover Medical School, Hannover, Germany
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Paredes O, Paredes K, Kawaguchi Y, Luque-Vasquez C, Chavez I, Celis J, Payet E, Ruiz E, Berrospi F. Clinicopathological factors associated with recurrence in patients undergoing resection of pancreatic solid pseudopapillary neoplasm. Discov Oncol 2021; 12:53. [PMID: 35201506 PMCID: PMC8777513 DOI: 10.1007/s12672-021-00451-4] [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: 10/11/2021] [Accepted: 11/17/2021] [Indexed: 11/06/2022] Open
Abstract
PURPOSE Solid pseudopapillary neoplasm (SPN) is an uncommon pathology with a low-grade malignancy. Surgery is the milestone treatment. Nevertheless, despite appropriate management, some patients present recurrence. Risk factors associated with recurrence are unclear. The objective was to identify the clinicopathological factors associated with recurrence in patients with SPN treated with pancreatic resection. METHODS Medical records of patients treated with pancreatic resection during 2006-2020 were evaluated. Patients with histological diagnosis of SPN were included. Survival analysis was performed to identify the clinicopathological factors related to recurrence. RESULTS Seventy-four patients were diagnosed with SPN; 70 (94.6%) patients were female, and the median age was 20 years old. The median tumor diameter was 7.9 cm. Multivisceral resection was performed in 9 (12.2%) patients. Four (5.4%) patients presented lymph node metastasis.R0 resection was achieved in all cases. Six (8%) patients presented recurrence and the liver was the most frequent recurrence site (n = 5).After a median follow-up of 40.2 months, 9 (12%) patients died. Five (6.8%) patients died of disease progression. The 1-3- and 5-year overall survival (OS) was 97.1%, 90.2% and 79.9%, respectively. The 1-3-and-5-year recurrence-free survival (RFS) was 98.4%, 89.9% and 87%, respectively. In the univariate Cox-regression analysis, age ≥ 28 years(HR = 8.61, 95% CI 1.1-73.8),tumor diameter ≥ 10 cm(HR = 9.3, 95% CI 1.12-79.6),invasion of adjacent organs (HR = 7.45, 95% CI 1.5-36.9), lymph node metastasis (pN +) (HR = 16.8, 95% CI 2.96-94.9) and, AJCC Stage III (HR = 10.1, 95% CI 1.2-90.9) were identified as predictors for recurrence. CONCLUSIONS SPN is more frequently diagnosed in young women with a good overall prognosis after an R0 surgical resection even with disease recurrence. Age ≥ 28 years, larger tumors ≥ 10 cm, invasion of adjacent organs, lymph node metastasis(pN +) and, AJCC Stage III were predictors factors of recurrence in resected SPN.
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Affiliation(s)
- Oscar Paredes
- Department of Abdominal Surgery, National Institute of Neoplastic Diseases INEN, Lima, Peru.
- , Montesquieu Street 277, Lima, Perú.
| | - Kori Paredes
- Department of Abdominal Surgery, National Institute of Neoplastic Diseases INEN, Lima, Peru
| | - Yoshikuni Kawaguchi
- Hepato-Biliary-Pancreatic Surgery Division, Department of Surgery, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan
| | - Carlos Luque-Vasquez
- Department of Abdominal Surgery, National Institute of Neoplastic Diseases INEN, Lima, Peru
| | - Iván Chavez
- Department of Abdominal Surgery, National Institute of Neoplastic Diseases INEN, Lima, Peru
| | - Juan Celis
- Department of Abdominal Surgery, National Institute of Neoplastic Diseases INEN, Lima, Peru
| | - Eduardo Payet
- Department of Abdominal Surgery, National Institute of Neoplastic Diseases INEN, Lima, Peru
| | - Eloy Ruiz
- Department of Abdominal Surgery, National Institute of Neoplastic Diseases INEN, Lima, Peru
| | - Francisco Berrospi
- Department of Abdominal Surgery, National Institute of Neoplastic Diseases INEN, Lima, Peru
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Kotb M, Fawzy O, Aboelela A, Aziz MA, Zaki I, Soliman M, Abouheba M. Solid pseudopapillary neoplasm of pancreas in a 14-year-old girl. JOURNAL OF PEDIATRIC SURGERY CASE REPORTS 2021. [DOI: 10.1016/j.epsc.2021.101991] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/29/2022] Open
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22
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Prediction of Recurrence of Completely Resected Pancreatic Solid Pseudopapillary Neoplasms in Pediatric Patients: A Single Center Analysis. CHILDREN-BASEL 2021; 8:children8080632. [PMID: 34438523 PMCID: PMC8391822 DOI: 10.3390/children8080632] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Subscribe] [Scholar Register] [Received: 06/28/2021] [Revised: 07/12/2021] [Accepted: 07/23/2021] [Indexed: 12/14/2022]
Abstract
Background: Many previous studies have investigated the risk factors for the recurrence of pancreatic solid pseudopapillary neoplasms (SPNs), although a consensus has not yet been reached, despite this effort. We aimed to identify the predictive factors for recurrence in patients with SPNs who underwent complete surgical resection of the tumor. Methods: We retrospectively analyzed the records of pediatric patients with SPNs who underwent surgical resection at a single center between 2001 and 2018. Results: During the study period, 47 patients with SPNs underwent radical resection of the tumor. The median age of the patients was 14 (8–18) years. R0 resection was confirmed in every case and none of the patients presented with systemic metastasis at the time of diagnosis. The median follow-up period was 53.1 (30.8–150.8) months. Of the 47 patients, only two (4.2%) experienced recurrence. Using comparative analysis, we found that some factors such as a large tumor size, peripancreatic tissue invasion, and capsule invasion did not increase the risk of recurrence of SPNs. Lymph node metastasis was the only significant factor for recurrence in our study (p = 0.043). Conclusion: During our single center analysis, we found that only lymph node metastasis was a predictive factor for recurrence of SPNs among patients who underwent complete tumor resection. Long-term follow-up is required to determine whether SPNs will recur if lymph node metastasis is observed after surgery. Furthermore, therapeutic benefits of routine lymphadenectomy or sentinel lymph node biopsy should be investigated in future studies to reduce the risk of recurrence in patients with SPNs.
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23
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Cai J, Chen H, Lu M, Zhang Y, Lu B, You L, Zhang T, Dai M, Zhao Y. Advances in the epidemiology of pancreatic cancer: Trends, risk factors, screening, and prognosis. Cancer Lett 2021; 520:1-11. [PMID: 34216688 DOI: 10.1016/j.canlet.2021.06.027] [Citation(s) in RCA: 193] [Impact Index Per Article: 48.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/26/2021] [Revised: 06/09/2021] [Accepted: 06/25/2021] [Indexed: 02/07/2023]
Abstract
Pancreatic cancer is a malignancy with poor prognosis and high mortality. The recent increase in pancreatic cancer incidence and mortality has resulted in an increased number of studies on its epidemiology. This comprehensive and systematic literature review summarizes the advances in the epidemiology of pancreatic cancer, including its epidemiological trends, risk factors, risk prediction models, screening modalities, and prognosis. The risk factors for pancreatic cancers can be categorized as those related to individual characteristics, lifestyle and environment, and disease status. Several prediction models for pancreatic cancer have been developed in populations with new-onset diabetes or a family history of pancreatic cancer; however, these models require further validation. Despite recent progress in pancreatic cancer screening, the quantity and quality of related studies are also unsatisfactory, especially with respect to the identification of high-risk populations and development of effective screening modality. Apart from the populations with familial genetic risk and those at a high risk of sporadic pancreatic cancer, risk factors such as new-onset diabetes may be a new direction for timely intervention. We hope this work will provide new ideas for further prevention and treatment of pancreatic cancer.
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Affiliation(s)
- Jie Cai
- Department of General Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, 100730, China
| | - Hongda Chen
- Office of Cancer Screening, National Cancer Center/National Clinical Research Center for Cancer/Cancer Hospital, Chinese Academy of Medical Sciences, Peking Union Medical College, Beijing, 100021, China
| | - Ming Lu
- Office of Cancer Screening, National Cancer Center/National Clinical Research Center for Cancer/Cancer Hospital, Chinese Academy of Medical Sciences, Peking Union Medical College, Beijing, 100021, China
| | - Yuhan Zhang
- Office of Cancer Screening, National Cancer Center/National Clinical Research Center for Cancer/Cancer Hospital, Chinese Academy of Medical Sciences, Peking Union Medical College, Beijing, 100021, China
| | - Bin Lu
- Office of Cancer Screening, National Cancer Center/National Clinical Research Center for Cancer/Cancer Hospital, Chinese Academy of Medical Sciences, Peking Union Medical College, Beijing, 100021, China
| | - Lei You
- Department of General Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, 100730, China
| | - Taiping Zhang
- Department of General Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, 100730, China
| | - Min Dai
- Office of Cancer Screening, National Cancer Center/National Clinical Research Center for Cancer/Cancer Hospital, Chinese Academy of Medical Sciences, Peking Union Medical College, Beijing, 100021, China.
| | - Yupei Zhao
- Department of General Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, 100730, China.
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Solid pseudo papillary tumor of the pancreas in a 7-year-old girl. JOURNAL OF PEDIATRIC SURGERY CASE REPORTS 2021. [DOI: 10.1016/j.epsc.2021.101828] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/20/2022] Open
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25
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Michael S, Kassam NM, Njau A, Sherman OA, Chuwa H, Surani S. A Rare Case of Solid Pseudopapillary Neoplasm of the Pancreas. Cureus 2021; 13:e14720. [PMID: 34079673 PMCID: PMC8159312 DOI: 10.7759/cureus.14720] [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] [Indexed: 01/13/2023] Open
Abstract
Solid pseudopapillary neoplasm of pancreas (SPN) is a rare entity. It is almost exclusively seen in females within the second and third decades of life with only small minority affecting children. Due to the paucity of the number of cases seen, the natural history of the disease is not fully understood. SPN tumors of the pancreas are usually found incidentally and usually have an excellent prognosis. We herein present a case of a 33-year-old lady diagnosed with SPN, who presented with abdominal fullness, two weeks post cesarean section.
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Affiliation(s)
- Steven Michael
- Surgery, Aga Khan University Medical College, Dar es Salaam, TZA
| | - Nadeem M Kassam
- Internal Medicine, Aga Khan University Medical College, Dar es Salaam, TZA
| | - Aidan Njau
- Surgery, Aga Khan University Medical College, Dar es Salaam, TZA
| | | | | | - Salim Surani
- Internal Medicine, Corpus Christi Medical Center, Corpus Christi, USA.,Internal Medicine, University of North Texas, Denton, USA
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26
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Brillantino C, Rossi E, Pirisi P, Gaglione G, Errico ME, Minelli R, Menna BF, Zeccolini R, Zeccolini M. Pseudopapillary solid tumour of the pancreas in paediatric age: description of a case report and review of the literature. J Ultrasound 2021; 25:251-257. [PMID: 33895967 DOI: 10.1007/s40477-021-00587-7] [Citation(s) in RCA: 10] [Impact Index Per Article: 2.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/03/2021] [Accepted: 04/13/2021] [Indexed: 12/01/2022] Open
Abstract
Pseudopapillary solid tumour of the pancreas is a rare neoplasm that mainly affects young women in the second and third decade of life and less frequently children; originates from the exocrine component of the pancreas; and is characterized by slow growth, low potential for malignancy, and excellent prognosis following complete surgical resection. The tumour often presents as an asymptomatic abdominal mass that is accidentally detected during radiological investigations performed for other reasons. In this article, we report the clinical case of a 10-year-old girl who came to our observation for pain in the left hypochondrium, which had arisen for a week following a trauma; the imaging methods revealed a voluminous expansive pancreatic formation in the abdomen; on histological examination, the mass was a solid pseudopapillary tumour. Furthermore, we present a review of the literature aimed at highlighting the salient features of this neoplasm in paediatric age.
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Affiliation(s)
- Carmela Brillantino
- Unit of Radiology and Ultrasound, Pausilipon Hospital, A.O.R.N. Santobono-Pausilipon, Naples, Italy
| | - Eugenio Rossi
- Unit of Radiology and Ultrasound, Pausilipon Hospital, A.O.R.N. Santobono-Pausilipon, Naples, Italy
| | - Pietro Pirisi
- Surgical Oncology Unit, Pausilipon Hospital, A.O.R.N. Santobono Pausilipon, Naples, Italy
| | - Giovanni Gaglione
- UOC Paediatric Surgery A.O.R.N. Santobono-Pausilipon, Santobono Hospital, Naples, Italy
| | - Maria E Errico
- Unit of Pathological Anatomy, Pausilipon Hospital, A.O.R.N. Santobono-Pausilipon, Naples, Italy
| | - Rocco Minelli
- Department of Life and Health "V. Tiberio", University of Molise, Francesco De Sanctis st 1, 86100, Campobasso, Italy.
| | - Biagio F Menna
- Department of Advanced Biomedical Sciences, University of Naples Federico II, via S. Pansini 5, 80131, Naples, Italy
| | | | - Massimo Zeccolini
- Unit of Radiology, Santobono Hospital, A.O.R.N. Santobono-Pausilipon, Naples, Italy
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Chen H, Huang Y, Yang N, Yan W, Yang R, Zhang S, Yang P, Li N, Feng Z. Solid-Pseudopapillary Neoplasm of the Pancreas: A 63-Case Analysis of Clinicopathologic and Immunohistochemical Features and Risk Factors of Malignancy. Cancer Manag Res 2021; 13:3335-3343. [PMID: 33883945 PMCID: PMC8055352 DOI: 10.2147/cmar.s304981] [Citation(s) in RCA: 11] [Impact Index Per Article: 2.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/02/2021] [Accepted: 03/26/2021] [Indexed: 01/17/2023] Open
Abstract
Purpose Solid-pseudopapillary neoplasm (SPN) of the pancreas, a rare tumor, has low malignant potential. However, some patients develop metastasis and recurrence after resection, with aggressive biological behaviors. This study aimed to explore the features and risk factors associated with the aggressive biological behaviors of SPNs. Patients and Methods We retrospectively analyzed the clinicopathological and long-term follow-up data of 63 patients diagnosed with SPN at the First Affiliated Hospital of Bengbu Medical College between January 2007 and February 2019. Results Sixty-three patients presented atypical clinical symptoms. The median tumor size was 7.0 cm (range, 2.4–17 cm), and imaging features were solid and cystic or solid tumors with uneven density. Frequent and diffuse nuclear LEF1 protein expression (94.2%) was observed with LEF1 having a higher sensitivity and specificity. Overall survival significantly correlated with tumor size, Ki-67 index, and lymph node metastasis (P < 0.05). Conclusion SPN is a rare low-grade malignancy with a specific pseudopapillary structure. LEF1 is an effective biomarker of SPNs. Although SPNs generally display indolent biological behavior, a large tumor size, high proliferation index, and lymph node metastasis may be risk factors for the aggressive behavior and poor prognosis of SPN.
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Affiliation(s)
- Hongchun Chen
- Department of Pathology, Bengbu Medical College, Bengbu, Anhui Province, 233000, People's Republic of China.,Department of Pathology, First Affiliated Hospital of Bengbu Medical College, Bengbu, Anhui Province, 233000, People's Republic of China
| | - Yuchen Huang
- Department of Pathology, Bengbu Medical College, Bengbu, Anhui Province, 233000, People's Republic of China.,Department of Pathology, First Affiliated Hospital of Bengbu Medical College, Bengbu, Anhui Province, 233000, People's Republic of China
| | - Ningning Yang
- Department of Pathology, Bengbu Medical College, Bengbu, Anhui Province, 233000, People's Republic of China.,Department of Pathology, First Affiliated Hospital of Bengbu Medical College, Bengbu, Anhui Province, 233000, People's Republic of China
| | - Wentian Yan
- Department of Pathology, Bengbu Medical College, Bengbu, Anhui Province, 233000, People's Republic of China.,Department of Pathology, First Affiliated Hospital of Bengbu Medical College, Bengbu, Anhui Province, 233000, People's Republic of China
| | - Ruxue Yang
- Department of Pathology, First Affiliated Hospital of Anhui Medical University, Hefei, Anhui Province, 230000, People's Republic of China
| | - Shan Zhang
- Department of Pathology, Second People's Hospital of Hefei, Hefei, Anhui Province, 230000, People's Republic of China
| | - Panpan Yang
- Department of Pathology, Second Affiliated Hospital of Anhui Medical University, Hefei, Anhui Province, 230000, People's Republic of China
| | - Nan Li
- Department of Pathology, Bengbu Medical College, Bengbu, Anhui Province, 233000, People's Republic of China.,Department of Pathology, First Affiliated Hospital of Bengbu Medical College, Bengbu, Anhui Province, 233000, People's Republic of China
| | - Zhenzhong Feng
- Department of Pathology, Bengbu Medical College, Bengbu, Anhui Province, 233000, People's Republic of China.,Department of Pathology, First Affiliated Hospital of Bengbu Medical College, Bengbu, Anhui Province, 233000, People's Republic of China
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28
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Kim MS, Park H, Lee S, Yoo SY, Cho SY, Lee SK, Jin DK. Clinical characteristics, treatment outcomes, and occurrence of diabetes mellitus after pancreatic resection of solid pseudopapillary tumor in children and adolescents: A single institution experience with 51 cases. Pancreatology 2021; 21:509-514. [PMID: 33509684 DOI: 10.1016/j.pan.2021.01.009] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/09/2020] [Revised: 12/25/2020] [Accepted: 01/15/2021] [Indexed: 12/11/2022]
Abstract
BACKGROUND Solid pseudopapillary tumors (SPTs) are rare, but they comprise the majority of pediatric pancreatic neoplasms. However, studies on these conditions in pediatric patients are lacking. The aim of this study was to investigate the clinical characteristics and treatment outcomes in children and adolescents with SPTs. METHODS This retrospective study included 51 patients with SPTs who had undergone pancreatic tumor resection before the age of 19 years at Samsung Medical Center in Korea (from November 1994 to August 2020). We investigated the postoperative outcomes. RESULTS Of the 51 patients with SPTs (female, 88.2%), the median age at diagnosis was 14 years (range, 8-19). The most common symptom was abdominal pain (60.8%), and 14 patients (27.5%) were asymptomatic. The median maximal tumor diameter was 7 cm (range, 1.4-14), and the pancreatic body and/or tail were involved in 68.6% of patients. The short-term complication rate was 21.5%, and the recurrence rate was 5.9%. New-onset diabetes mellitus (NODM) occurred in four patients. CONCLUSIONS The ideal treatment for SPTs is complete resection of the tumor; however, long-term postoperative complications including NODM should be monitored carefully, particularly in children and adolescents.
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Affiliation(s)
- Min-Sun Kim
- Department of Pediatrics, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Republic of Korea
| | - Hyojung Park
- Department of Pediatrics, Seongnam Citizens Medical Center, Gyeonggi-do, Republic of Korea
| | - Sanghoon Lee
- Department of Pediatric Surgery, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Republic of Korea
| | - So Young Yoo
- Department of Radiology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Republic of Korea
| | - Sung Yoon Cho
- Department of Pediatrics, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Republic of Korea.
| | - Suk-Koo Lee
- Department of Pediatric Surgery, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Republic of Korea
| | - Dong-Kyu Jin
- Department of Pediatrics, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Republic of Korea
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29
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Uğuz A, Ünalp ÖV, Akpınar G, Karaca CA, Oruç N, Nart D, Yılmaz F, Aydın A, Çoker A. Solid pseudopapillary neoplasms of the pancreas: Case series with a review of the literature. TURKISH JOURNAL OF GASTROENTEROLOGY 2021; 31:930-935. [PMID: 33626007 DOI: 10.5152/tjg.2020.19227] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 01/17/2023]
Abstract
BACKGROUND/AIMS The solid pseudopapillary neoplasms are quite rare tumors of the pancreas, comprising roughly 1-2% of all pancreatic neoplasms. It has a low malignant potential and usually affects young females. Despite increasing number of articles in the last decade, there is still debate on the pathogenesis, malignant potential and optimal surgical strategy for the solid pseudopapillary neoplasms. MATERIALS AND METHODS Medical recordings of 326 patients who were operated due to pancreatic mass were retrospectively analyzed. Patient demographics, presenting symptoms, surgical and pathologic characteristics of the tumor, postsurgical course, long-term survival, and other relevant data were extracted from patients' charts. RESULTS Majority of the patients were female in consistency with the classic data in the literature. All the patients underwent curative intent resections. Tumors were commonly localized in the tail of the pancreas making distal pancreatectomy the most commonly performed surgical procedure. Mean tumor diameter was 5.8 centimeters with tumor sizes ranging from 1 to 19 cm. CONCLUSION The solid pseudopapillary neoplasms of the pancreas is a rare tumor with low malignant potential, which is more common in females of reproductive age, with abdominal pain being their most common presentation. The short-term outcomes in patients following surgical R0 resection are excellent. However, proximal placement of the tumor and female gender may have slightly worse prognosis. We hope that our findings from a series of patients represent a contribution to the existing literature on SPN, and authors declare their willingness to provide further details for future meta-analyses.
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Affiliation(s)
- Alper Uğuz
- Department of General Surgery, Ege University School of Medicine, İzmir, Turkey
| | - Ömer Vedat Ünalp
- Department of General Surgery, Ege University School of Medicine, İzmir, Turkey
| | - Göksever Akpınar
- Department of General Surgery, University of Health Sciences Tepecik Training and Research Hospital, İzmir, Turkey
| | - Can Avni Karaca
- Department of General Surgery, Izmir University of Economics School of Medicine
| | - Nevin Oruç
- Department of Gastroeneterology, Ege University School of Medicine, İzmir, Turkey
| | - Deniz Nart
- Department of Pathology, Ege University School of Medicine, İzmir, Turkey
| | - Funda Yılmaz
- Department of Pathology, Ege University School of Medicine, İzmir, Turkey
| | - Ahmet Aydın
- Department of Gastroeneterology, Ege University School of Medicine, İzmir, Turkey
| | - Ahmet Çoker
- Department of Gastroeneterology, Ege University School of Medicine, İzmir, Turkey
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30
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Lemoine A, Asmandar S, Boutroux H, Tounian P, Ducou Le Pointe H, Coulomb A, Irtan S. Extrapancreatic primary solid pseudopapillary tumor in the gastric antrum: Case report. Pediatr Blood Cancer 2020; 67:e28415. [PMID: 32779872 DOI: 10.1002/pbc.28415] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/15/2020] [Revised: 04/28/2020] [Accepted: 04/29/2020] [Indexed: 01/01/2023]
Affiliation(s)
- Anaïs Lemoine
- Department of Pediatric Nutrition and Gastroenterology, Trousseau Hospital, APHP, Sorbonne University, Paris, France
| | - Safaa Asmandar
- Department of Pathological Anatomy and Cytology, Trousseau Hospital, APHP, Sorbonne University, Paris, France
| | - Hélène Boutroux
- Department of Pediatric Hematology and Oncology, APHP, Trousseau Hospital, Paris, France
| | - Patrick Tounian
- Department of Pediatric Nutrition and Gastroenterology, Trousseau Hospital, APHP, Sorbonne University, Paris, France
| | - Hubert Ducou Le Pointe
- Department of Pediatric Imaging, APHP, Sorbonne University, Trousseau Hospital, Paris, France
| | - Aurore Coulomb
- Department of Pathological Anatomy and Cytology, Trousseau Hospital, APHP, Sorbonne University, Paris, France
| | - Sabine Irtan
- Department of Visceral and Neonatal Pediatric Surgery, APHP, Sorbonne University, Trousseau Hospital, Paris, France
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31
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Clinicopathological findings of solid pseudopapillary neoplasm in a 10-year-old girl presenting with pruritus, a case report and concise review. HUMAN PATHOLOGY: CASE REPORTS 2020. [DOI: 10.1016/j.ehpc.2020.200449] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/23/2022] Open
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32
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Shah I, Mehta H, Anklesaria A, Kadkhodayan K. Solid Pseudopapillary Tumor of the Pancreas in a Patient With Sarcoidosis: A Rare Combination. Cureus 2020; 12:e8340. [PMID: 32617215 PMCID: PMC7325336 DOI: 10.7759/cureus.8340] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/07/2023] Open
Abstract
Sarcoidosis is a chronic granulomatous disease that is characterized by the formation of non-caseating granulomas, predominantly involving the lung and lymph nodes. Over the years, sarcoidosis has been associated with a high risk of malignancy. Solid pseudopapillary tumor of the pancreas is an uncommon pancreatic tumor with a 15% malignant potential. Ours is an interesting case of a 34-year-old patient who was found to have a pancreatic mass and incidental mediastinal lymphadenopathy on imaging, initially raising concern for metastatic pancreatic cancer. However, she was later diagnosed to have an isolated solid pseudopapillary tumor of the pancreas in association with concurrent sarcoidosis.
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Affiliation(s)
- Ishani Shah
- Internal Medicine, Creighton University School of Medicine, St. Joseph's Hospital and Medical Center, Phoenix, USA
| | - Harsh Mehta
- Internal Medicine, Saint Barnabas Medical Center, Livingston, USA
| | - Ava Anklesaria
- Gastroenterology, Maimonides Medical Center, Brooklyn, USA
| | - Kambiz Kadkhodayan
- Gastroenterology, Creighton University School of Medicine, St. Joseph's Hospital and Medical Center, Phoenix, USA
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33
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Ihara T, Nomura O, Mori T, Shimojima N. Early Diagnosis of a Pediatric Pancreatic Tumor by Abdominal Physical Examination: A Case Report. J Emerg Med 2020; 58:e211-e214. [PMID: 32317196 DOI: 10.1016/j.jemermed.2020.03.025] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/30/2019] [Revised: 02/19/2020] [Accepted: 03/18/2020] [Indexed: 11/25/2022]
Abstract
BACKGROUND Detecting a neoplasm in pediatric patients with acute abdominal pain is difficult, especially when there is no palpable mass. Ihara's maneuver, which allows the physician to apply manual pressure directly on the pancreatic body, is a useful palpation method of diagnosing acute pancreatitis in children. We report a case of solid pseudopapillary neoplasm of the pancreas (SPNP) detected by Ihara's maneuver. CASE REPORT An otherwise healthy, 15-year-old male visited our emergency department with acute abdominal pain and nausea. His vital signs were appropriate for his age. A physical examination denied peritoneal signs, but Ihara's maneuver induced strong tenderness. His serum amylase and lipase were normal. A contrast-enhanced computed tomography scan revealed a well-defined, 2.2-cm, nonenhanced mass in the pancreatic tail. Laparoscopic distal pancreatectomy was performed, and the diagnosis of SPNP was confirmed. The patient was well postoperatively without any metastasis. SPNP is a rare neoplasm with low malignant potential. Although it typically occurs in young females, it has also been reported in children. The early diagnosis of SPNP is usually challenging because most patients do not have specific symptoms or laboratory findings. In the present case, the SPNP was difficult to detect by palpation because of its size, but Ihara's maneuver induced strong tenderness of the pancreas and led to a diagnosis. WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS?: Ihara's maneuver has the potential to enable early diagnosis not only of pancreatitis but also of pancreatic tumors, such as SPNP.
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Affiliation(s)
- Takateru Ihara
- Division of Pediatric Emergency Medicine, Department of Pediatric Emergency and Critical Care Medicine, Tokyo, Japan
| | - Osamu Nomura
- Division of Pediatric Emergency Medicine, Department of Pediatric Emergency and Critical Care Medicine, Tokyo, Japan; Department of Emergency and Disaster Medicine, Hirosaki University, Aomori, Japan
| | - Takaaki Mori
- Division of Pediatric Emergency Medicine, Department of Pediatric Emergency and Critical Care Medicine, Tokyo, Japan
| | - Naoki Shimojima
- Department of Surgery, Tokyo Metropolitan Children's Medical Center, Tokyo, Japan
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Wright MJ, Javed AA, Saunders T, Zhu Y, Burkhart RA, Yu J, He J, Cameron JL, Makary MA, Wolfgang CL, Weiss MJ. Surgical Resection of 78 Pancreatic Solid Pseudopapillary Tumors: a 30-Year Single Institutional Experience. J Gastrointest Surg 2020; 24:874-881. [PMID: 31073801 DOI: 10.1007/s11605-019-04252-7] [Citation(s) in RCA: 16] [Impact Index Per Article: 3.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/14/2019] [Accepted: 04/25/2019] [Indexed: 02/07/2023]
Abstract
BACKGROUND Solid pseudopapillary tumors (SPTs) are rare, benign tumors of the pancreas that present as heterogeneous masses. We sought to evaluate the short- and long-term outcomes of surgical resected SPTs. Patients managed via initial surveillance were compared to those who underwent upfront resection. METHODS A prospectively maintained institutional database was used to identify patients who underwent surgical resection for a SPT between 1988 and 2018. Data on clinicopathological features and outcomes were collected and analyzed. RESULTS Seventy-eight patients underwent surgical resection for SPT during the study period. The mean age was 34.0 ± 14.6 years and a majority were female (N = 67, 85.9%) and white (N = 46, 58.9%). Thirty patients (37.9%) were diagnosed incidentally. Imaging-based presumed diagnosis was SPT in 49 patients (62.8%). A majority were located in the body or tail of the pancreas (N = 47, 60.3%), and 48 patients (61.5%) underwent a distal pancreatectomy. The median tumor size was 4.0 cm (IQR, 3.0-6.0), nodal disease was present in three patients (3.9%), and R0 resection was performed in all patients. No difference was observed in clinicopathological features and outcomes between patients who were initially managed via surveillance and those who underwent upfront resection. None of the patients under surveillance had nodal disease or metastasis at the time of resection; however, one of them developed recurrence of disease 95.1 months after resection. At a median follow-up of 36.1 months (IQR, 8.1-62.1), 77 (%) patients were alive and one patient (1.3%) had a recurrence of disease at 95.1 months after resection and subsequently died due to disease. CONCLUSIONS SPTs are rare pancreatic tumors that are diagnosed most frequently in young females. While a majority are benign and have an indolent course, malignant behavior has been observed. Surgical resection can result in exceptional outcomes.
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Affiliation(s)
- Michael J Wright
- The John L. Cameron Division of Hepatobiliary and Pancreatic Surgery, The Johns Hopkins Hospital, Baltimore, MD, USA
| | - Ammar A Javed
- The John L. Cameron Division of Hepatobiliary and Pancreatic Surgery, The Johns Hopkins Hospital, Baltimore, MD, USA
| | - Tyler Saunders
- The John L. Cameron Division of Hepatobiliary and Pancreatic Surgery, The Johns Hopkins Hospital, Baltimore, MD, USA
| | - Yayun Zhu
- The John L. Cameron Division of Hepatobiliary and Pancreatic Surgery, The Johns Hopkins Hospital, Baltimore, MD, USA
| | - Richard A Burkhart
- The John L. Cameron Division of Hepatobiliary and Pancreatic Surgery, The Johns Hopkins Hospital, Baltimore, MD, USA
| | - Jun Yu
- The John L. Cameron Division of Hepatobiliary and Pancreatic Surgery, The Johns Hopkins Hospital, Baltimore, MD, USA
| | - Jin He
- The John L. Cameron Division of Hepatobiliary and Pancreatic Surgery, The Johns Hopkins Hospital, Baltimore, MD, USA
| | - John L Cameron
- The John L. Cameron Division of Hepatobiliary and Pancreatic Surgery, The Johns Hopkins Hospital, Baltimore, MD, USA
| | - Martin A Makary
- The John L. Cameron Division of Hepatobiliary and Pancreatic Surgery, The Johns Hopkins Hospital, Baltimore, MD, USA
| | - Christopher L Wolfgang
- The John L. Cameron Division of Hepatobiliary and Pancreatic Surgery, The Johns Hopkins Hospital, Baltimore, MD, USA
| | - Matthew J Weiss
- The John L. Cameron Division of Hepatobiliary and Pancreatic Surgery, The Johns Hopkins Hospital, Baltimore, MD, USA. .,Pancreas Cancer Multidisciplinary Clinic, Liver Cancer Multidisciplinary Clinic, Surgical Oncology Fellowship, Miller Coulson Academy of Clinical Excellence, Johns Hopkins University, 600 N. Wolfe St. / Blalock 685, Baltimore, MD, 21287, USA.
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35
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Farhat W, Ammar H, Amine Said M, Mizouni A, Bouazzi A, Abdessaied N, Ben Mabrouk M, Ben Ali A. Solid pseudopapillary neoplasm of the pancreas: a report of 10 cases and literature review. ANZ J Surg 2020; 90:1683-1688. [PMID: 31989788 DOI: 10.1111/ans.15701] [Citation(s) in RCA: 12] [Impact Index Per Article: 2.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/26/2019] [Accepted: 01/07/2020] [Indexed: 12/13/2022]
Abstract
BACKGROUND Solid pseudopapillary tumour of the pancreas (SPTP) is a rare pancreatic tumour characterized by a non-specific clinical presentations and vague radiologic features. The aim of this study is to identify these tumours from other pancreatic neoplasms because complete resection is curative in most cases and provides long-term survival. METHODS A retrospective analysis of patients operated for SPTP between January 2000 and December 2018 was conducted. The collected data included age, gender, clinical findings, laboratory tests, radiological findings, anatomopathological examination, immunohistochemistry results, surgical treatment, mortality, morbidity and recurrence. RESULTS Ten cases of SPTP have been diagnosed between January 2000 and December 2018 representing 5.1% of all pancreatic tumours operated during this period (male/female: 2/8; median age 41.2 years; range 19-78 years). The most common symptom was abdominal pain and physical examination was normal in four of 10 cases. The most common tumour localization was the tail of the pancreas. The main tumour size was 7.2 cm (range 2-15 cm). One patient had abdominal disseminated disease. Surgical interventions were distal pancreatectomy in five cases, enucleation in one case, cephalic duodeno-pancreatectomy in two cases, central pancreatectomy in one case and pancreatic biopsy in one case. Only one patient received adjuvant chemotherapy. During follow-up, one patient died after 12 months and another developed unique hepatic metastasis that was resected. CONCLUSION Although it is delayed in diagnosis, the overall prognosis of these tumours remains good even with local recurrence and metastasis. Complete surgical resection is the treatment of choice even in cases of recurrence.
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Affiliation(s)
- Waad Farhat
- Department of Gastrointestinal Surgery, Sahloul Hospital, Sousse, Tunisia
| | - Houssem Ammar
- Department of Gastrointestinal Surgery, Sahloul Hospital, Sousse, Tunisia
| | - Mohamed Amine Said
- Department of Gastrointestinal Surgery, Sahloul Hospital, Sousse, Tunisia
| | - Abdelkader Mizouni
- Department of Gastrointestinal Surgery, Sahloul Hospital, Sousse, Tunisia
| | - Amal Bouazzi
- Department of Gastrointestinal Surgery, Sahloul Hospital, Sousse, Tunisia
| | - Nihed Abdessaied
- Department of Pathology, Farhat Hached Hospital, University of Sousse, Sousse, Tunisia
| | | | - Ali Ben Ali
- Department of Gastrointestinal Surgery, Sahloul Hospital, Sousse, Tunisia
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36
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Guo T, Wang L, Xie P, Zhang Z, Yu Y. Diagnosis and Surgical Treatment and Pathological Findings of Solid Pseudopapillary Tumor of the Pancreas: A Single-Institution Experience. Cancer Manag Res 2020; 12:581-588. [PMID: 32158262 PMCID: PMC6986403 DOI: 10.2147/cmar.s238527] [Citation(s) in RCA: 8] [Impact Index Per Article: 1.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/17/2019] [Accepted: 01/14/2020] [Indexed: 12/17/2022] Open
Abstract
Purpose To investigate the diagnosis, surgical treatment, and pathology of solid pseudopapillary tumors of the pancreas in our institution. Patients and Methods We retrospectively analyzed the demographic details, clinical features, imaging findings, and pathological findings of 87 patients with a confirmed diagnosis of solid pseudopapillary tumors of the pancreas (SPTP) and underwent surgery in Tongji Hospital of Tongji Medical College, over a period of 8 years from 2011 to 2018. Results Our study involved a total of 87 patients (16 males and 71 females) with a mean age of 31.3±13.1 years (range: 10–61 years). The main compliant was abdominal pain or discomfort (n=49) and the median tumor size was 58.6±31.7 mm (range: 16–156mm). Tumors were located in the head (27 patients, 31%), the neck (13 patients, 15%), and the body and tail (47 patients, 54%). There were no significant differences between the patients in terms of sex, age, or tumor location. Partial pancreatectomy was performed in 79 patients, enucleation in six patients, and total pancreatectomy in two patients. R0 resection was achieved in 86 patients. The postoperative morbidity was 36.8%, and the main complication was pancreatic fistula. Pathological examination and immunohistochemical markers were used to provide a final diagnosis. The main follow-up period was 46 months (range: 13–97 months). At the end of the follow-up period, 86 patients were alive and had not experienced recurrence; one patient was lost to follow-up. Conclusion The accurate diagnosis of SPTP is vital. Our data showed that surgical resection is safe and associated with low morbidity and mortality rates. Pathological findings can play an important role in diagnosis and long-term survival.
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Affiliation(s)
- Tong Guo
- Department of Biliary-Pancreatic Surgery, Affiliated Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan 430030, Hubei, People's Republic of China
| | - Lu Wang
- Department of Biliary-Pancreatic Surgery, Affiliated Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan 430030, Hubei, People's Republic of China
| | - Peng Xie
- Department of Biliary-Pancreatic Surgery, Affiliated Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan 430030, Hubei, People's Republic of China
| | - Zhiwei Zhang
- Department of Biliary-Pancreatic Surgery, Affiliated Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan 430030, Hubei, People's Republic of China
| | - Yahong Yu
- Department of Biliary-Pancreatic Surgery, Affiliated Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan 430030, Hubei, People's Republic of China
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Xu M, Li XJ, Zhang XE, Pan FS, Tan Y, Huang TY, Hong Y, Huang GL, Xie XY. Application of Contrast-Enhanced Ultrasound in the Diagnosis of Solid Pseudopapillary Tumors of the Pancreas: Imaging Findings Compared With Contrast-Enhanced Computed Tomography. JOURNAL OF ULTRASOUND IN MEDICINE : OFFICIAL JOURNAL OF THE AMERICAN INSTITUTE OF ULTRASOUND IN MEDICINE 2019; 38:3247-3255. [PMID: 31179567 DOI: 10.1002/jum.15036] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Received: 12/30/2018] [Revised: 04/28/2019] [Accepted: 05/13/2019] [Indexed: 06/09/2023]
Abstract
OBJECTIVES The purpose of this study was to assess the characterization and usefulness of contrast-enhanced ultrasound (CEUS) for diagnosing solid pseudopapillary tumors of the pancreas (SPTP) and compare the enhancement patterns with contrast-enhanced computed tomography (CECT). METHODS Forty-three SPTP lesions proved by pathologic findings in 42 patients examined with CEUS and CECT were included in this study. The enhancement characteristics and typical CEUS features of the tumors were investigated. These characteristics were compared according to lesion sizes. The enhancement patterns of CEUS were compared with CECT. RESULTS The most common enhancement levels of SPTP in the early phase and late phase for CEUS were isoenhancement (19 of 43 [44.2%]) and hypoenhancement (32 of 43 [74.4%]), respectively. The 4 most common enhancement patterns were hypo-hypo (16 of 43 [37.2%]), iso-iso (11 of 43 [25.6%]), hyper-hypo (8 of 43 [18.6%]), and iso-hypo (8 of 43 [18.6%]) enhancement. For the 43 SPTP lesions, typical CEUS features such as lesion membrane, intralesional vessel, and intralesional compartmentalization enhancements were detected in 30 (69.8%), 27 (62.8%), and 10 (23.2%) cases. Compared with CECT, isoenhancement during the early phase and hypoenhancement during the late phase were the most common imaging characteristics of CEUS. CONCLUSIONS Lesion membrane, intralesional vessel, and intralesional compartmentalization enhancements are typical CEUS features of SPTP, especially for large lesions (lesion size ≥3.0 cm). Isoenhancement during the early phase and hypoenhancement during the late phase are the most common imaging characteristics of CEUS and CECT, making CEUS a viable alternative diagnostic method that is noninvasive.
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Affiliation(s)
- Ming Xu
- Department of Medical Ultrasound, Institute of Diagnostic and Interventional Ultrasound, First Affiliated Hospital of Sun Yat-Sen University, Guangzhou, China
- Department of Radiology, University of Texas Southwestern Medical Center, Dallas, Texas, USA
| | - Xiao-Ju Li
- Department of Medical Ultrasound, Institute of Diagnostic and Interventional Ultrasound, First Affiliated Hospital of Sun Yat-Sen University, Guangzhou, China
| | - Xiao-Er Zhang
- Department of Medical Ultrasound, Institute of Diagnostic and Interventional Ultrasound, First Affiliated Hospital of Sun Yat-Sen University, Guangzhou, China
| | - Fu-Shun Pan
- Department of Medical Ultrasound, Institute of Diagnostic and Interventional Ultrasound, First Affiliated Hospital of Sun Yat-Sen University, Guangzhou, China
| | - Yang Tan
- Department of Medical Ultrasound, Institute of Diagnostic and Interventional Ultrasound, First Affiliated Hospital of Sun Yat-Sen University, Guangzhou, China
| | - Tong-Yi Huang
- Department of Medical Ultrasound, Institute of Diagnostic and Interventional Ultrasound, First Affiliated Hospital of Sun Yat-Sen University, Guangzhou, China
| | - Yu Hong
- University of Texas at Dallas, Richardson, Texas, USA
| | - Guang-Liang Huang
- Department of Medical Ultrasound, Institute of Diagnostic and Interventional Ultrasound, First Affiliated Hospital of Sun Yat-Sen University, Guangzhou, China
| | - Xiao-Yan Xie
- Department of Medical Ultrasound, Institute of Diagnostic and Interventional Ultrasound, First Affiliated Hospital of Sun Yat-Sen University, Guangzhou, China
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van Huijgevoort NCM, Del Chiaro M, Wolfgang CL, van Hooft JE, Besselink MG. Diagnosis and management of pancreatic cystic neoplasms: current evidence and guidelines. Nat Rev Gastroenterol Hepatol 2019; 16:676-689. [PMID: 31527862 DOI: 10.1038/s41575-019-0195-x] [Citation(s) in RCA: 140] [Impact Index Per Article: 23.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Accepted: 07/31/2019] [Indexed: 12/11/2022]
Abstract
Pancreatic cystic neoplasms (PCN) are a heterogeneous group of pancreatic cysts that include intraductal papillary mucinous neoplasms, mucinous cystic neoplasms, serous cystic neoplasms and other rare cystic lesions, all with different biological behaviours and variable risk of progression to malignancy. As more pancreatic cysts are incidentally discovered on routine cross-sectional imaging, optimal surveillance for patients with PCN is becoming an increasingly common clinical problem, highlighting the need to balance cancer prevention with the risk of (surgical) overtreatment. This Review summarizes the latest developments in the diagnosis and management of PCN, including the quality of available evidence. Also discussed are the most important differences between the PCN guidelines from the American Gastroenterological Association, the International Association of Pancreatology and the European Study Group on Cystic Tumours of the Pancreas, including diagnostic and follow-up strategies and indications for surgery. Finally, new developments in the management of patients with PCN are addressed.
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Affiliation(s)
- Nadine C M van Huijgevoort
- Department of Gastroenterology and Hepatology, Amsterdam Gastroenterology and Metabolism, Amsterdam UMC, University of Amsterdam, Amsterdam, Netherlands
| | - Marco Del Chiaro
- Department of Surgery, University of Colorado Anschutz Medical Campus, Aurora, Colorado, USA
| | - Christopher L Wolfgang
- Department of Surgery, The Sol Goldman Pancreatic Cancer Research Center, The Johns Hopkins University School of Medicine, Baltimore, MD, USA
| | - Jeanin E van Hooft
- Department of Gastroenterology and Hepatology, Amsterdam Gastroenterology and Metabolism, Amsterdam UMC, University of Amsterdam, Amsterdam, Netherlands
| | - Marc G Besselink
- Department of Surgery, Cancer Center Amsterdam, Amsterdam UMC, University of Amsterdam, Amsterdam, Netherlands.
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ElHaddad A, Gasparella P, Castellani C, Singer G, Sorantin E, Zach K, Till H. Laparoscopic Distal Pancreatectomy of a Solid Pseudopapillary Tumor (SPT) Achieves Long-Term Oncologic Safety and Multiorgan Preservation. European J Pediatr Surg Rep 2019; 7:e58-e62. [PMID: 31440438 PMCID: PMC6703992 DOI: 10.1055/s-0039-1693999] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/10/2019] [Accepted: 06/18/2019] [Indexed: 02/06/2023] Open
Abstract
The oncological safety of a laparoscopic approach for solid pseudopapillary tumors (SPTs) of the pancreas remains a matter of debate. We present the long-term follow-up of an adolescent girl with an SPT in the pancreatic tail. A multimodality workup including magnetic resonance imaging (MRI) revealed a complex, spherical mass of 4.4 cm × 3.6 cm × 4 cm most likely located in the pancreatic tail. All routine laboratory investigations and tumor markers were within normal limits (alpha fetoprotein [AFP], cancer antigen 125 [CA125], CA 19-9, carcinoembryonic antigen [CEA], adrenocorticotropic hormone [ACTH]). Diagnostic laparoscopy was performed to verify the origin of the tumor in the pancreatic tail. In a three-port technique the tumor was mobilized of the splenic vessels until a distal pancreatectomy could be completed. Histopathological examination confirmed the complete resection of a low-grade malignant SPT. The postoperative course was unremarkable. Regular pediatric oncological follow-up examinations for 3 years, including MRI every 6 months, ruled out recurrence and confirmed preservation of splenic and pancreatic functions. While data about the technical feasibility of a laparoscopic approach to pancreatic SPT are already available, this pediatric case report adds a long-term oncological and functional success to the available literature.
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Affiliation(s)
- Ahmed ElHaddad
- Department of Paediatric and Adolescent Surgery, Medical University Graz, Graz, Austria.,Department of Pediatric Surgery, Section of Pediatric Surgery, Tanta University, Tanta, Egypt
| | - Paolo Gasparella
- Department of Paediatric and Adolescent Surgery, Medical University Graz, Graz, Austria
| | - Christoph Castellani
- Department of Paediatric and Adolescent Surgery, Medical University Graz, Graz, Austria
| | - Georg Singer
- Department of Paediatric and Adolescent Surgery, Medical University Graz, Graz, Austria
| | - Erich Sorantin
- Department of Paediatric and Adolescent Surgery, Medical University Graz, Graz, Austria
| | - Klara Zach
- Department of Paediatric and Adolescent Surgery, Medical University Graz, Graz, Austria
| | - Holger Till
- Department of Paediatric and Adolescent Surgery, Medical University Graz, Graz, Austria
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40
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Yalçın B, Yağcı‐Küpeli B, Ekinci S, Orhan D, Oğuz B, Varan A, Kutluk T, Akyüz C. Solid pseudopapillary neoplasm of the pancreas in children: Hacettepe experience. ANZ J Surg 2019; 89:E236-E240. [DOI: 10.1111/ans.15111] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/13/2018] [Revised: 12/15/2018] [Accepted: 01/25/2019] [Indexed: 12/14/2022]
Affiliation(s)
- Bilgehan Yalçın
- Department of Pediatric OncologyHacettepe University Faculty of Medicine Ankara Turkey
| | - Begül Yağcı‐Küpeli
- Department of Pediatric OncologyHacettepe University Faculty of Medicine Ankara Turkey
- Department of Pediatric Hematology/OncologyAdana City Education and Research Hospital, Sağlık Bilimleri University Adana Turkey
| | - Saniye Ekinci
- Department of Pediatric SurgeryHacettepe University Faculty of Medicine Ankara Turkey
| | - Diclehan Orhan
- Department of PathologyHacettepe University Faculty of Medicine Ankara Turkey
| | - Berna Oğuz
- Department of RadiologyHacettepe University Faculty of Medicine Ankara Turkey
| | - Ali Varan
- Department of Pediatric OncologyHacettepe University Faculty of Medicine Ankara Turkey
| | - Tezer Kutluk
- Department of Pediatric OncologyHacettepe University Faculty of Medicine Ankara Turkey
| | - Canan Akyüz
- Department of Pediatric OncologyHacettepe University Faculty of Medicine Ankara Turkey
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Stutchfield BM, Nayar M, Penman ID. Pancreatic cystic lesions: risk stratification and management based on recent guidelines. Frontline Gastroenterol 2019; 10:182-187. [PMID: 31205661 PMCID: PMC6540278 DOI: 10.1136/flgastro-2018-101076] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/06/2018] [Revised: 01/08/2019] [Accepted: 01/09/2019] [Indexed: 02/04/2023] Open
Abstract
Pancreatic cystic lesions (PCLs) can present complex diagnostic and management challenges with uncertainty as to the most appropriate investigations, interventions and surveillance. Guidelines have been developed to aid decision making, including the European Study Group, American College of Gastroenterology and International Study Group guidelines. This paper presents issues relating to risk stratification and the appropriate management of patients with PCLs, reviewing these recently published guidelines. While there are similarities across these expert guidelines, there are notable differences in terms of features associated with increased risk of malignant transformation, the most appropriate imaging modality and timing of interval imaging. Where variations exist, this reflects differing interpretations of a limited evidence base, and decision making will likely evolve further as experience with these guidelines develops.
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Affiliation(s)
| | - Manu Nayar
- Department of Gastroenterology, The Freeman Hospital, Newcastle, UK
| | - Ian D Penman
- Centre for Liver and Digestive Disorders, Royal Infirmary of Edinburgh, Edinburgh, UK
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Wang F, Meng Z, Li S, Zhang Y, Wu H. Prognostic value of progesterone receptor in solid pseudopapillary neoplasm of the pancreas: evaluation of a pooled case series. BMC Gastroenterol 2018; 18:187. [PMID: 30547767 PMCID: PMC6295102 DOI: 10.1186/s12876-018-0914-8] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/10/2018] [Accepted: 11/28/2018] [Indexed: 02/07/2023] Open
Abstract
Background The role of progesterone receptor (PR) has been reported in a series of pancreatic cysts. However, the relationship between PR and prognosis of solid pseudopapillary neoplasm of the pancreas (SPNP) has not been elucidated so far. The aim of our study was to evaluate the prognostic value of PR in SPNP. Methods A total of 76 patients with SPNP treated in our institution from January 2012 to December 2017 were included. Demographic parameters, laboratory data, pathologic information and clinical outcomes were analyzed by the use of survival analysis. In addition, a pooled case series was performed to evaluate the results. Results The institutional data included 76 patients (17 male and 59 female) ranging from 8 to 90 years (median, 30 years) in age. Kaplan-Meier survival analysis confirmed negative PR result was significantly associated with poorer disease-free survival (DFS) and disease-specific survival (DSS) (both P < 0.001). In the pooled analysis, a total of 62 studies comprising 214 patients with SPNP were included. After multivariable cox analysis, negative PR result remained an independent prognostic factor for SPNP (DFS HR: 14.50, 95% CI: 1.98–106.05, P = 0.008; DSS HR: 9.15, 95% CI: 1.89–44.17, P = 0.006). Conclusion Our results indicated the role of PR in predicting adverse outcome of patients with SPNP and negative PR result may serve as a potential prognostic factor. Electronic supplementary material The online version of this article (10.1186/s12876-018-0914-8) contains supplementary material, which is available to authorized users.
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Affiliation(s)
- Feiyang Wang
- Department of General Surgery, Shanghai Jiaotong University Affiliated First People's Hospital, Shanghai, 200080, China.,Department of Pancreatic Surgery, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, 430022, China
| | - Zibo Meng
- Department of Pancreatic Surgery, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, 430022, China
| | - Shoukang Li
- Department of Thoracic Surgery, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, 430022, China
| | - Yushun Zhang
- Department of Pancreatic Surgery, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, 430022, China
| | - Heshui Wu
- Department of Pancreatic Surgery, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, 430022, China.
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Bartolini I, Bencini L, Bernini M, Farsi M, Calistri M, Annecchiarico M, Moraldi L, Coratti A. Robotic enucleations of pancreatic benign or low-grade malignant tumors: preliminary results and comparison with robotic demolitive resections. Surg Endosc 2018; 33:2834-2842. [PMID: 30421079 DOI: 10.1007/s00464-018-6576-3] [Citation(s) in RCA: 7] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/05/2018] [Accepted: 11/02/2018] [Indexed: 12/27/2022]
Abstract
BACKGROUND The incidental detection of benign to low-grade malignant small pancreatic neoplasms increased in the last decades. The surgical management of these patients is still under debate. The aim of this paper is to evaluate the safety and feasibility of robotic enucleations and to compare the outcomes with non-parenchymal sparing robotic resections. METHODS The study included a total of 25 patients. Nine of them underwent a robotic enucleation (EN Group) and 16 patients received a robotic demolitive resection (DR Group). Perioperative and medium-term outcomes were compared between the two groups. RESULTS Patients' baseline characteristics were similar in the two groups except for presence of symptoms and tumor size, due to the inclusion criteria. Operative time was significantly shorter and postoperative results were better for EN group, including a significant shorter hospitalization (5 vs. 8 days, p = 0.027), reduced pancreatic leaks (22% vs. 50%, p = 0.287) and a better preservation of glandular function (100% vs. 62.5%, p = 0.066). Mortality rate was zero in both groups, with all patients free from disease at a median follow-up of 18 months. CONCLUSIONS The risks of under/overtreatment remain still unavoidable for benign to low-grade malignant small pancreatic neoplasms. Simple enucleation should be performed whenever oncological appropriate, to achieve the best postoperative outcomes. The adoption of robotic technique might widen the indications for parenchymal sparing, minimally invasive surgery.
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Affiliation(s)
- Ilenia Bartolini
- Department of Oncology, Division of Surgical Oncology and Robotics, AOU Careggi, Largo Brambilla 3, 50134, Florence, Italy. .,Department of Surgery and Translational Medicine, Hepatobiliary Surgery Unit, University of Florence-AOU Careggi, Largo Brambilla 3, 50134, Florence, Italy.
| | - Lapo Bencini
- Department of Oncology, Division of Surgical Oncology and Robotics, AOU Careggi, Largo Brambilla 3, 50134, Florence, Italy
| | - Marco Bernini
- Department of Oncology, Division of Oncologic and Reconstructive Breast Surgery, Breast Unit, AOU Careggi, Largo Brambilla 3, 50134, Florence, Italy
| | - Marco Farsi
- Department of Oncology, Division of Surgical Oncology and Robotics, AOU Careggi, Largo Brambilla 3, 50134, Florence, Italy
| | - Massimo Calistri
- Department of Oncology, Division of Surgical Oncology and Robotics, AOU Careggi, Largo Brambilla 3, 50134, Florence, Italy
| | - Mario Annecchiarico
- Department of Oncology, Division of Surgical Oncology and Robotics, AOU Careggi, Largo Brambilla 3, 50134, Florence, Italy
| | - Luca Moraldi
- Department of Oncology, Division of Surgical Oncology and Robotics, AOU Careggi, Largo Brambilla 3, 50134, Florence, Italy
| | - Andrea Coratti
- Department of Oncology, Division of Surgical Oncology and Robotics, AOU Careggi, Largo Brambilla 3, 50134, Florence, Italy
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Bender AM, Thompson ED, Hackam DJ, Cameron JL, Rhee DS. Solid Pseudopapillary Neoplasm of the Pancreas in a Young Pediatric Patient: A Case Report and Systematic Review of the Literature. Pancreas 2018; 47:1364-1368. [PMID: 30325866 DOI: 10.1097/mpa.0000000000001183] [Citation(s) in RCA: 20] [Impact Index Per Article: 2.9] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/26/2022]
Abstract
Solid pseudopapillary neoplasms (SPNs) are the most common pediatric pancreatic tumor; however, most data in children are extrapolated from adults. This study describes a young presentation of SPN in a 5-year-old girl and presents a comprehensive systematic review of the literature regarding SPNs in children. A systematic review was performed using PubMed and Embase for all articles in English using predetermined search terms, including "solid pseudopapillary neoplasm" and "pediatric" and historical terms for SPN. A total of 523 pediatric patients were identified in 135 articles. Eighty-three percent of patients were female, and median age was 13.6 years. Abdominal pain was the most frequent presenting symptom (78%), and median tumor size was 8.2 cm. The pancreatic head was involved in 46% of cases. Computed tomographic scan was the most common imaging modality (87%), and 61% were diagnosed by fine needle aspiration. Surgical resection was reported in 507 patients, with a complication rate of 21.1% reported in 393 patients. Only 3.8% received adjuvant therapy, and 6.7% had recurrent disease. Solid pseudopapillary neoplasms of the pancreas are rare tumors in childhood. Male sex and pancreatic head involvement are seen more often in children than in adults. Surgery remains the mainstay of treatment with excellent results.
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Affiliation(s)
| | | | - David J Hackam
- Surgery, Johns Hopkins University School of Medicine, Baltimore, MD
| | - John L Cameron
- Surgery, Johns Hopkins University School of Medicine, Baltimore, MD
| | - Daniel S Rhee
- Surgery, Johns Hopkins University School of Medicine, Baltimore, MD
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46
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Abstract
OBJECTIVES Prognostic factors for solid pseudopapillary neoplasms (SPN) of the pancreas have been incompletely defined in the literature. We aimed to use the National Cancer Database to describe survival and characterize prognostic factors in patients with pancreatic pseudopapillary neoplasms. METHODS We identified 304 patients with pancreatic SPN diagnosed between 2004 and 2012 using the National Cancer Database. All patients were included in the survival analysis. Kaplan-Meier method, χ, and log-rank tests were used for statistical analysis. Cox proportional hazard regression model was used for multivariate analysis. RESULTS The median age at diagnosis was 36.5 years. Eighty-five percent of patients were female, 69% were white, and 25% were black. Metastases were observed in 5% of patients. Median tumor size was 5.1 cm. Ninety-two percent of patients underwent primary tumor resection. At 60 months, 98% of patients who underwent resection were alive, and 40% who did not undergo resection were alive. On multivariate analysis, female sex, resection of primary tumor, and absence of metastasis were correlated with improved survival. CONCLUSIONS Patients with SPN who undergo resection have an excellent survival at 5 years. Surgery should be considered in all patients with this diagnosis.
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47
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Ercelep O, Ozdemir N, Turan N, Topcu TO, Uysal M, Tanriverdi O, Demirci U, Taskoylu BY, Urakcı Z, Duran AO, Aksoy A, Menekse S, Ozcelik M, Gumus M. Retrospective evaluation of patients diagnosed solid pseudopapillary neoplasms of the pancreas. Curr Probl Cancer 2018; 43:27-32. [PMID: 30104029 DOI: 10.1016/j.currproblcancer.2018.06.014] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/17/2018] [Accepted: 06/30/2018] [Indexed: 12/29/2022]
Abstract
PURPOSE Solid pseudopapillary neoplasm (SPN) is a rare, low-grade neoplasm with excellent prognosis. In this study, we evaluated clinicopathological characteristics of patients diagnosed with SPN retrospectively. METHODS This is a retrospective study intended to characterize patients with the diagnosis of SPN between 2005 and 2015. Clinicopathological features, recurrence rate, and overall survival of 28 patients were recorded. Malignant SPN criteria were defined as the presence of distant metastasis (developed at diagnosis or during follow up) or lymph node involvement. RESULTS The mean age at diagnosis was 42 (range: 17-41). Among patients, 82% (n = 23) were female and 17.9% (n = 5) were male. The mean size of tumor was 5.81 cm (range: 2-15). The mean follow up period was 55.6 months, 1-year survival was 96.5% and 5-year survival rate was 88%. A total of 25 patients were alive at the end of follow-up period and 3 of the patients became exitus due to disease. Two patients had a metastatic presentation in livers at the diagnosis and metastasis developed in 3 patients during follow-up (liver of 1 patient, peritoneum in 1 patient and liver and peritoneum in 1 patient). The reason of admission was headache in 68% patients. The type of operation was frequently subtotal pancreatectomy (n = 11, 39.3%) and distal pancreatectomy (n = 10, 35.7%). Tumors were located frequently in body and tail regions (n = 18, 64.3%) and the number of patients with malignant criteria was 6 (21.4%). Although the mean age of malignant patients was significantly higher than benign patients (P = 0.046), there was no significant difference between 2 groups in terms of gender, tumor size, capsule invasion, perineural invasion, vascular invasion, and margin status. CONCLUSION SPN is a rarely seen tumor with low malignity potential. Surgical resection provides long-term survival rate even in local invasion or metastasis conditions.
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Affiliation(s)
- Ozlem Ercelep
- Department of Medical Oncology, Dr. Lutfi Kirdar Kartal Education and Research Hospital, Istanbul, Turkey.
| | - Nuriye Ozdemir
- Department of Medical Oncology, Ankara Numune Education and Research Hospital, Ankara, Turkey
| | - Nedim Turan
- Department of Medical Oncology, Faculty of Medicine, Gazi University, Ankara, Turkey
| | - Turkan Ozturk Topcu
- Department of Medical Oncology, Faculty of Medicine, Karadeniz Technical University, Trabzon, Turkey
| | - Mukremin Uysal
- Department of Medical Oncology, Faculty of Medicine, Afyon Kocatepe University, Afyon, Turkey
| | - Ozgur Tanriverdi
- Department of Medical Oncology, Faculty of Medicine, Mugla Sıtkı Kocaman University, Mugla, Turkey
| | - Umut Demirci
- Department of Medical Oncology, Ankara Yurtaslan Oncology Hospital, Ankara, Turkey
| | - Burcu Yapar Taskoylu
- Department of Medical Oncology, Faculty of Medicine, Pamukkale University, Denizli, Turkey
| | - Zuhat Urakcı
- Department of Medical Oncology, Faculty of Medicine, Dicle University, Diyarbakır, Turkey
| | - Ayse Ocak Duran
- Department of Medical Oncology, Faculty of Medicine, Kayseri Erciyes University, Kayseri, Turkey
| | - Asude Aksoy
- Department of Medical Oncology, Faculty of Medicine, Malatya Inonu University, Malatya, Turkey
| | - Serkan Menekse
- Department of Medical Oncology, Faculty of Medicine, Celal Bayar University, Manisa, Turkey
| | - Melike Ozcelik
- Department of Medical Oncology, Dr. Lutfi Kirdar Kartal Education and Research Hospital, Istanbul, Turkey
| | - Mahmut Gumus
- Department of Medical Oncology, Faculty of Medicine, Bezmi Alem Vakif University, Istanbul, Turkey
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You L, Yang F, Fu DL. Prediction of malignancy and adverse outcome of solid pseudopapillary tumor of the pancreas. World J Gastrointest Oncol 2018; 10:184-193. [PMID: 30079144 PMCID: PMC6068856 DOI: 10.4251/wjgo.v10.i7.184] [Citation(s) in RCA: 29] [Impact Index Per Article: 4.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/16/2018] [Revised: 04/23/2018] [Accepted: 05/23/2018] [Indexed: 02/05/2023] Open
Abstract
Since solid pseudopapillary tumor of the pancreas (SPTP) was officially classified by the World Health Organization in 1996, SPTP has recently received special attention in the literature. Studies have shown that SPTP is a heterogeneous tumor, with a small percentage of patients harboring aggressive behaviors. However, criteria for malignancy grade in SPTP have not been well established. The prognosis of SPTP is generally good, with cases having a chance for long-term survival even with recurrence and/or metastasis after surgical resection. The current American Joint Committee on Cancer/Union for International Cancer Control tumor, node, metastasis staging system is not specific to SPTP. The lack of a predictive staging classification that accurately describes the heterogeneity of this disease hinders meaningful research into optimal individualized therapy. Here we summarize and discuss the associated factors proposed for appraisal of the malignant potential and adverse outcome of SPTP.
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Affiliation(s)
- Li You
- Department of Pancreatic Surgery, Pancreatic Disease Institute, Huashan Hospital, Shanghai Medical College, Fudan University, Shanghai 200040, China
| | - Feng Yang
- Department of Pancreatic Surgery, Pancreatic Disease Institute, Huashan Hospital, Shanghai Medical College, Fudan University, Shanghai 200040, China
| | - De-Liang Fu
- Department of Pancreatic Surgery, Pancreatic Disease Institute, Huashan Hospital, Shanghai Medical College, Fudan University, Shanghai 200040, China
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Wang P, Wei J, Wu J, Xu W, Chen Q, Gao W, Jiang K, Miao Y. Diagnosis and treatment of solid-pseudopapillary tumors of the pancreas: A single institution experience with 97 cases. Pancreatology 2018; 18:415-419. [PMID: 29548800 DOI: 10.1016/j.pan.2017.12.012] [Citation(s) in RCA: 22] [Impact Index Per Article: 3.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/13/2017] [Revised: 12/18/2017] [Accepted: 12/20/2017] [Indexed: 12/11/2022]
Abstract
BACKGROUND/OBJECTIVES We evaluated the diagnoses and surgical management of solid pseudopapillary tumors of the pancreas (SPTP) in a single center setting. METHODS Demographic details, clinical presentations, imaging features, surgical strategies, and pathological findings of 97 consecutive patients who underwent surgery for pathologically confirmed SPTP between 2008 and 2016 were analyzed retrospectively. RESULTS A total of 97 patients with SPTP accounted for 2.15% of total pancreatic surgeries (N = 4508). The mean age at diagnosis was 31.6 ± 13.92 years (range: 7-83 years), and males:females were 85:12. Chief complaints were abdominal pain or discomfort (n = 50). The mean transverse tumor diameter was 53.2 ± 2.76 mm (range: 14-170 mm). Tumors were localized to the pancreatic head (38.1%; 37/97), neck (12.4%; 12/97), body and tail (49.5%; 48/97). There were no significant differences in age of onset, presenting symptoms, and sites between males and females. A significant difference was found in the maximum diameter of SPTP across different age groups. Partial pancreatectomy was performed for patient with tumor ≥ 5 cm, and enucleation was performed when tumor was smaller than 5 cm. Pancreatic fistula was the main complication. Immunohistochemical markers for endocrine, exocrine and epithelial factors were used for a final diagnosis. During a median follow-up of 54 months (range: 7-121 months), 84 patients were alive without evidence of recurrence, and 13 patients were lost to follow-up. CONCLUSION SPTP surgical resection is a safe procedure with low morbidity and mortality, which is also effective even in the presence of invasiveness and metastases. Accurate initial diagnoses and follow up are essential.
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Affiliation(s)
- Peng Wang
- Pancreas Center, The First Affiliated Hospital of Nanjing Medical University, Nanjing, 210029, China
| | - Jishu Wei
- Pancreas Center, The First Affiliated Hospital of Nanjing Medical University, Nanjing, 210029, China
| | - Junli Wu
- Pancreas Center, The First Affiliated Hospital of Nanjing Medical University, Nanjing, 210029, China
| | - Wenbin Xu
- Pancreas Center, The First Affiliated Hospital of Nanjing Medical University, Nanjing, 210029, China
| | - Qiuyang Chen
- Pancreas Center, The First Affiliated Hospital of Nanjing Medical University, Nanjing, 210029, China
| | - Wentao Gao
- Pancreas Center, The First Affiliated Hospital of Nanjing Medical University, Nanjing, 210029, China
| | - Kuirong Jiang
- Pancreas Center, The First Affiliated Hospital of Nanjing Medical University, Nanjing, 210029, China
| | - Yi Miao
- Pancreas Center, The First Affiliated Hospital of Nanjing Medical University, Nanjing, 210029, China.
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Solid Pseudopapillary Neoplasm of the Pancreas in Children and Adults: A National Study of 369 Patients. J Pediatr Hematol Oncol 2018; 40:e233-e236. [PMID: 29240036 DOI: 10.1097/mph.0000000000001049] [Citation(s) in RCA: 16] [Impact Index Per Article: 2.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/13/2022]
Abstract
BACKGROUND Solid pseudopapillary neoplasm (SPN) of the pancreas is a rare tumor in children, with current evidence limited to single-center studies. We examined treatment and clinical outcomes for pediatric and adult SPN with a national data set. METHODS The 2004 to 2013 National Cancer Data Base was queried to identify all patients diagnosed with SPN. The cohort was stratified by age (pediatric and adult) defined as below 18 years and 18 years and above, respectively. Baseline characteristics and unadjusted outcomes were compared. RESULTS We identified 21 pediatric and 348 adult patients with SPN. Both groups displayed similar demographic composition. Patients were commonly female (90.5% [pediatric] vs. 85.9% [adult], P=0.56) and white (66.7% vs. 68.3%, P=0.74). Tumor location was similar between adults and children. Median tumor size was similar between children and adults (5.9 vs. 4.9 cm, P=0.41). Treatment strategies did not vary between groups. Partial pancreatectomy was the most common resection strategy (71.4% vs. 53.1%, P=0.80). Both groups experienced low mortality (0.0% vs. 0.7% at 5 y, P=0.31). CONCLUSIONS This study provides the largest comparison of pediatric and adult SPN to date. Children with SPN have similar disease severity at presentation, receive similar treatments, and demonstrate equivalent postoperative outcomes compared with their adult counterparts.
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