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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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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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Gao Y, Guo F, Lu Z, Xi C, Wei J, Jiang K, Miao Y, Wu J, Chen J. Perioperative safety and prognosis following parenchyma-preserving surgery for solid pseudopapillary neoplasm of the pancreas. World J Surg Oncol 2023; 21:119. [PMID: 37004027 PMCID: PMC10064731 DOI: 10.1186/s12957-023-03003-y] [Citation(s) in RCA: 6] [Impact Index Per Article: 3.0] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/19/2022] [Accepted: 03/22/2023] [Indexed: 04/03/2023] Open
Abstract
BACKGROUND/OBJECTIVES To evaluate perioperative safety and outcome of parenchyma-preserving pancreatectomy and risk factors of metastasis and recurrence for patients with solid pseudopapillary neoplasm (SPN). METHODS Demographic data, operative and pathological parameter, follow-up data of patients with SPN undergoing their first operation were collected in our single center from May 2016 to October 2021 and compared between regular pancreatectomy group and parenchyma-preserving surgery group. Risk factors for metastasis and recurrence were investigated. RESULTS A total of 194 patients were included, 154 of whom were female and the average age of all patients was 33 years old. Most patients were asymptomatic, with the most common complaint being abdominal pain or discomfort. Of them, 62 patients underwent parenchyma-preserving pancreatectomy including middle segment pancreatectomy and enucleation, and 132 patients underwent regular pancreatectomy including pancreaticoduodenectomy, distal pancreatectomy and total pancreatectomy. Patients in the parenchyma-preserving surgery group had a shorter duration of operation, less intraoperative bleeding, and decreased risk of combined organ removal and blood transfusion, with no statistical significance yet. The two groups exhibited a similar incidence of postoperative complications including grade B and C pancreatic fistula, delayed gastric emptying, postoperative pancreatic hemorrhage, and other complications, as well as radiological intervention, relaparotomy and the length of postoperative hospital stay. There were no perioperative deaths. All the patients, except 18 of those who discontinued follow-up, were alive with a median follow-up time of 31 months. Three patients in the regular pancreatectomy group were observed to have liver metastasis, and no metastasis was observed in the parenchyma-preserving surgery group. Significant risk factors for tumor metastasis and recurrence were tumor size, angioinvasion, and nerve infiltration. CONCLUSIONS Parenchyma-preserving surgery did not significantly increase the frequency of perioperative complications or recurrence and might be preferable if comprehensive conditions allow.
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Affiliation(s)
- Yong Gao
- Pancreas Center, The First Affiliated Hospital with Nanjing Medical University, Nanjing, Jiangsu, 210029, People's Republic of China
| | - Feng Guo
- Pancreas Center, The First Affiliated Hospital with Nanjing Medical University, Nanjing, Jiangsu, 210029, People's Republic of China
| | - Zipeng Lu
- Pancreas Center, The First Affiliated Hospital with Nanjing Medical University, Nanjing, Jiangsu, 210029, People's Republic of China
| | - Chunhua Xi
- Pancreas Center, The First Affiliated Hospital with Nanjing Medical University, Nanjing, Jiangsu, 210029, People's Republic of China
| | - Jishu Wei
- Pancreas Center, The First Affiliated Hospital with Nanjing Medical University, Nanjing, Jiangsu, 210029, People's Republic of China
| | - Kuirong Jiang
- Pancreas Center, The First Affiliated Hospital with Nanjing Medical University, Nanjing, Jiangsu, 210029, People's Republic of China
| | - Yi Miao
- Pancreas Center, The First Affiliated Hospital with Nanjing Medical University, Nanjing, Jiangsu, 210029, People's Republic of China
- Pancreas Center, The Affiliated BenQ Hospital of Nanjing Medical University, Nanjing, Jiangsu, 210029, People's Republic of China
| | - Junli Wu
- Pancreas Center, The First Affiliated Hospital with Nanjing Medical University, Nanjing, Jiangsu, 210029, People's Republic of China.
| | - Jianmin Chen
- Pancreas Center, The First Affiliated Hospital with Nanjing Medical University, Nanjing, Jiangsu, 210029, People's Republic of China.
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Wu J, Tian X, Liu B, Li C, Hao C. Features and Treatment of Peritoneal Metastases from Solid Pseudopapillary Neoplasms of the Pancreas. Med Sci Monit 2018. [PMID: 29524354 PMCID: PMC5858737 DOI: 10.12659/msm.909363] [Citation(s) in RCA: 8] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/11/2022] Open
Abstract
Background Solid pseudopapillary neoplasms (SPNs) of the pancreas may present widespread peritoneal metastases, but the treatment of this malignancy has not been well described and requires further investigation. Material/Methods Four cases of SPN with significant peritoneal metastases in our department were operated and retrospectively summarized after long-term follow-up. Eight more cases of peritoneal metastatic SPN from the PubMed database were also included in the analysis. Results Peritoneal metastases of SPNs have different gross features. The benign nodules were tenacious and well encapsulated, while the malignant nodules were soft and prone to slow bleeding. However, neither of these nodules invaded the small intestines or mesentery. Of the 12 disseminated cases, 7 had history of primary tumor rupture, whereas the others had tumors malignant in nature. A total of 14 surgical events were documented, including 3 complete cytoreductive surgeries (CCRS), 9 cytoreductive surgeries (CRS), and 2 debulking surgeries. After follow-up ranging from 0.3 to 6.1 years, the results of the Fisher’s exact test showed no difference between CCRS and CRS in treating either low-grade or high-grade malignant SPNs (P=0.257 and P=0.203, respectively). For all cases of SPN with peritoneal metastases, the CCRS procedure could significantly improve tumor-free survival (TFS) compared to the CRS procedure (P=0.046). Conclusions SPN rupture could cause significant peritoneal metastases, and either disruption or biopsy of these lesions should be avoided. Peritoneal metastases from SPNs vary both in gross features and biological mechanisms. CCRS may offer optimal therapeutic outcomes and longer TFS for individuals with significant peritoneal metastases of SPNs.
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Affiliation(s)
- Jianhui Wu
- Key laboratory of Carcinogenesis and Translational Research (Ministry of Education/Beijing), Department of Hepato-Pancreato-Biliary Surgery, Peking University Cancer Hospital and Institute, Beijing, China (mainland)
| | - Xiuyun Tian
- Key laboratory of Carcinogenesis and Translational Research (Ministry of Education/Beijing), Department of Hepato-Pancreato-Biliary Surgery, Peking University Cancer Hospital and Institute, Beijing, China (mainland)
| | - Bonan Liu
- Key laboratory of Carcinogenesis and Translational Research (Ministry of Education/Beijing), Department of Hepato-Pancreato-Biliary Surgery, Peking University Cancer Hospital and Institute, Beijing, China (mainland)
| | - Chengpeng Li
- Key laboratory of Carcinogenesis and Translational Research (Ministry of Education/Beijing), Department of Hepato-Pancreato-Biliary Surgery, Peking University Cancer Hospital and Institute, Beijing, China (mainland)
| | - Chunyi Hao
- Key laboratory of Carcinogenesis and Translational Research (Ministry of Education/Beijing), Department of Hepato-Pancreato-Biliary Surgery, Peking University Cancer Hospital and Institute, Beijing, China (mainland)
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Morikawa T, Onogawa T, Maeda S, Takadate T, Shirasaki K, Yoshida H, Ishida K, Motoi F, Naitoh T, Rikiyama T, Katayose Y, Egawa S, Unno M. Solid pseudopapillary neoplasms of the pancreas: an 18-year experience at a single Japanese Institution. Surg Today 2012; 43:26-32. [PMID: 23114787 DOI: 10.1007/s00595-012-0345-z] [Citation(s) in RCA: 37] [Impact Index Per Article: 2.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/27/2011] [Accepted: 09/07/2011] [Indexed: 12/13/2022]
Abstract
PURPOSE This study investigated the clinicopathological features and surgical management of solid pseudopapillary neoplasms at a single institution in Japan. METHODS Seventeen patients (the largest series in Japan) those underwent surgery for pathologically confirmed solid pseudopapillary neoplasms were retrospectively reviewed. RESULTS Sixteen patients were women and their mean age was 34.1 years. Most patients were asymptomatic (n = 11), and the average tumor diameter was 51.8 mm. The most common imaging characteristic was tumors of solid and cystic type (n = 10), which were most commonly located in the pancreatic body (n = 7). All patients underwent surgical exploration, i.e., distal pancreatectomies in 7 patients (laparoscopically performed in 2); middle pancreatectomies, 4; pancreaticoduodenectomies, 4; enucleation, 1; and liver resection, 1. No surgical mortalities occurred, and postsurgical complications occurred in 9 patients. Four patients had malignant tumors. One patient with liver metastases experienced recurrence, which was well controlled by paclitaxel. The remaining patients were disease free at a median follow-up of 51 months. CONCLUSIONS Solid pseudopapillary neoplasms can be treated by complete tumor resection with limited resection or a minimally invasive approach when applicable. The combination of surgical resection and chemotherapy may therefore prolong survival, even in malignant cases.
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Affiliation(s)
- Takanori Morikawa
- Division of Hepato-Biliary-Pancreatic Surgery, Tohoku University Graduate School of Medicine, 1-1 Seiryo-cho, Aoba-ku, Sendai 980-8574, Japan
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Cavallini A, Alvise C, Butturini G, Giovanni B, Daskalaki D, Despoina D, Salvia R, Roberto S, Melotti G, Gianluigi M, Piccoli M, Micaela P, Bassi C, Claudio B, Pederzoli P, Paolo P. Laparoscopic pancreatectomy for solid pseudo-papillary tumors of the pancreas is a suitable technique; our experience with long-term follow-up and review of the literature. Ann Surg Oncol 2010; 18:352-7. [PMID: 20848223 DOI: 10.1245/s10434-010-1332-5] [Citation(s) in RCA: 30] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/14/2010] [Indexed: 02/06/2023]
Abstract
BACKGROUND Solid pseudopapillary tumors (SPTs) are rare pancreatic neoplasms of low malignant potential that occur mainly in young women. Only 17 cases of SPT treated laparoscopically have been published in the literature and long-term follow-up data are still lacking. METHODS Retrospective analysis of ten patients (8 women, 2 men; mean age, 25.4 years) (DS: 12.1; minimum 11, maximum 51) who underwent laparoscopic distal pancreatectomy with a definitive histological diagnosis of SPT. Long-term follow-up data were collected. RESULTS The average tumor size was 43.8 mm (minimum 20, maximum 65 mm). The mean operative time was 177.5 minutes (DS: 53.7; minimum 120, maximum 255). In all, five patients underwent distal splenopancreatectomy; five patients underwent spleen-preserving distal pancreatectomy of whom three with splenic vessel preservation and two with the Warshaw technique. The conversion rate was nil and no case of perioperative mortality was recorded. The mean hospital stay was 7 days (DS: 2.7; minimum 4, maximum 12). Six patients had an uneventful postoperative course and four had postoperative complications. Two of them underwent reoperation, and the other two had nonsurgical complications. After a median follow-up of 47 (range, 5-98) months, all patients were alive and disease-free. CONCLUSIONS Laparoscopic pancreatic resection is a safe and feasible procedure that could become the treatment of choice for patients affected by pancreatic SPT. Distal pancreatectomy should be performed, if possible, with spleen-preserving technique, especially in young patients. To avoid metastatic spread, laparoscopic or laparotomic biopsy should not be performed in patients affected by SPT.
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Affiliation(s)
- Alvise Cavallini
- Surgical Department, Chirurgia Generale B-Policlinico GB Rossi-Borgo Roma, Verona University, Verona, Italy
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Soloni P, Cecchetto G, Dall'igna P, Carli M, Toffolutti T, Bisogno G. Management of unresectable solid papillary cystic tumor of the pancreas. A case report and literature review. J Pediatr Surg 2010; 45:e1-6. [PMID: 20438906 DOI: 10.1016/j.jpedsurg.2010.02.045] [Citation(s) in RCA: 20] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/14/2009] [Revised: 02/09/2010] [Accepted: 02/11/2010] [Indexed: 01/15/2023]
Abstract
Pancreatic solid papillary cystic tumor is a rare neoplasm with an excellent prognosis if surgical excision is complete. We report on a case and review 47 more cases extracted from the published literature to assess the treatment options when solid papillary cystic tumor is considered unresectable. Chemotherapy and radiotherapy were beneficial in a limited number of patients, but therapeutic decisions must be made bearing in mind that patients may be long-term survivors without any treatment because of the tumor's slow growth.
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Affiliation(s)
- Pietro Soloni
- Division of Hematology/Oncology, Department of Pediatrics, University Hospital of Padova, 35128 Padova, Italy
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Fais PO, Carricaburu E, Sarnacki S, Berrebi D, Orbach D, Baudoin V, de Lagausie P. Is laparoscopic management suitable for solid pseudo-papillary tumors of the pancreas? Pediatr Surg Int 2009; 25:617-21. [PMID: 19479267 DOI: 10.1007/s00383-009-2388-9] [Citation(s) in RCA: 31] [Impact Index Per Article: 1.9] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Accepted: 05/15/2009] [Indexed: 01/27/2023]
Abstract
PURPOSE Solid pseudo-papillary tumors (SPT) are rare pancreatic neoplasms of low-malignant potential occurring mainly in young women. The purpose of this report is to describe our experience with laparoscopic management of these tumors with 4-year follow-up. METHODS Three children with SPT were admitted to two hospitals in Paris, France, between February 2000 and December 2006. Diagnosis or treatment was carried out using laparoscopic techniques (biopsy and resection in one case and biopsy only in two). Long-term follow-up data were collected. RESULTS All three patients presented recurrences within 3 years after resection, i.e., disseminated peritoneal recurrence in two patients and local recurrence in one. The two patients with peritoneal recurrences were treated by surgical resection and chemotherapy. The patient with local recurrence could not be treated due to contraindicating local factors. All three patients were alive at the time of this writing. CONCLUSION This is the first report describing long-term follow-up after laparoscopic management of SPT. All three patients developed recurrences. These poor results contrast sharply with the low risk of local or disseminated recurrence after open laparotomy without chemotherapy that has been considered as the treatment of choice up to now. Recurrences after laparoscopic management may have been due to diffusion of tumor cells caused by gas insufflation especially during biopsy. Laparoscopic biopsy should not be performed in patients presenting SPT.
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Affiliation(s)
- P O Fais
- Department of Pediatric Surgery, Timone Enfants Hospital, 264 Bld Saint Pierre, 13385 Marseille Cedex 05, France
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Yang F, Fu DL, Jin C, Long J, Yu XJ, Xu J, Ni QX. Clinical experiences of solid pseudopapillary tumors of the pancreas in China. J Gastroenterol Hepatol 2008; 23:1847-1851. [PMID: 18752561 DOI: 10.1111/j.1440-1746.2008.05526.x] [Citation(s) in RCA: 37] [Impact Index Per Article: 2.2] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 01/08/2023]
Abstract
AIMS To discuss the clinical experiences of solid pseudopapillary tumors (SPTs) of the pancreas by summarizing clinical information of patients with this disease in China. METHODS Chinese literature concerning SPTs of the pancreas published between January 1996 and October 2006 were retrospectively reviewed and analyzed. RESULTS A total of 390 cases had been reported, among which 47 were men, with a female to male ratio of 7.30:1. Mean age of the patients was 25.3 years old, more than 50 per cent were between 10 and 24 years. The mean diameter of the tumor was 8.4 cm (range, 2 cm-25 cm). There was no significant difference in patient age and tumor size between male and female. Major clinical presentations included abdominal pain or discomfort, and palpable abdominal masses, however, nearly one third of all patients were asymptomatic. The rate of pre-operative misdiagnosis was rather high. Those who tested positive to metastases or invasions, 14.4% of the patients were diagnosed as malignant SPTs. Sex, age, symptoms, tumor size and tumor markers were not significant clinical factors to predict SPTs with malignant potential. Surgical procedures mainly included pancreatoduodenectomy, distal pancreatectomy and local resection. Three patients developed local recurrence, and one patient developed hepatic metastasis, all within four years after tumor resection. Five patients with malignant SPTs died due to tumor progression within 25 months after surgery. CONCLUSIONS Surgical resection is the most effective means for curing this rare tumor. Despite metastasis, a good satisfactory effect could be achieved by surgical debulking. At least 4-yearly follow-up is mandatory for all patients undergoing surgical resection.
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Affiliation(s)
- Feng Yang
- Department of Surgery, Pancreatic Disease Institute, Huashan Hospital, Shanghai Medical College, Fudan University, Shanghai, China
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Jhala N, Siegal GP, Jhala D. Large, clear cytoplasmic vacuolation: an under-recognized cytologic clue to distinguish solid pseudopapillary neoplasms of the pancreas from pancreatic endocrine neoplasms on fine-needle aspiration. Cancer 2008; 114:249-54. [PMID: 18484644 DOI: 10.1002/cncr.23595] [Citation(s) in RCA: 45] [Impact Index Per Article: 2.6] [Reference Citation Analysis] [Abstract] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/23/2022]
Abstract
BACKGROUND Solid pseudopapillary neoplasm (SPN) and pancreatic endocrine neoplasm (PEN) are uncommon neoplasms that demonstrate characteristic cytologic features. It is also known that both these tumors may share similar morphologic changes. These features not uncommonly pose significant diagnostic challenge for unsuspecting cytopathologists. In the current study, the authors report that recognition of clear cytoplasmic vacuoles in endoscopic ultrasound-guided fine-needle aspiration (EUS-FNA) samples from SPN serves as a useful clue that can distinguish this tumor from PEN. METHODS The cytologic features from 5 SPN and 20 PEN cases were evaluated. Both Diff-Quik and Papanicolaou stains from these cases were examined. A Fisher exact test of probability was performed to determine differences in the individual cytologic features noted in these 2 tumor types. RESULTS The results demonstrated that pseudopapillary groups (P = .004); metachromatic matrix material (P = .004); nuclear membrane irregularity (P = .004); and large, clear cytoplasmic vacuoles (P = .001) are noted significantly more frequently in SPN. The authors also demonstrated that large, clear cytoplasmic vacuoles can serve as a powerful cytologic clue for the suspicion of SPN over PEN when there is a paucity of papillary groups within the smears. Large, clear cytoplasmic vacuoles, however, were noted only in Diff-Quik-stained smears, but not in Papanicolaou-;stained smears. CONCLUSIONS The results of the current study highlight that large, clear cytoplasmic vacuoles can serve as a critical clue with which to distinguish SPN from PEN in diagnostically challenging cases.
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Affiliation(s)
- Nirag Jhala
- Department of Pathology, University of Alabama at Birmingham, 19th Street and 5th Avenue South, Birmingham, AL 35249, USA.
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Sperti C, Berselli M, Pasquali C, Pastorelli D, Pedrazzoli S. Aggressive behaviour of solid-pseudopapillary tumor of the pancreas in adults: a case report and review of the literature. World J Gastroenterol 2008; 14:960-965. [PMID: 18240360 PMCID: PMC2687069 DOI: 10.3748/wjg.14.960] [Citation(s) in RCA: 77] [Impact Index Per Article: 4.5] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/02/2007] [Revised: 12/17/2007] [Indexed: 02/06/2023] Open
Abstract
Solid-pseudopapillary tumor (SPT) is a rare neoplasm of the pancreas that usually occurs in young females. It is generally considered a low-grade malignant tumor that can remain asymptomatic for several years. The occurrence of infiltrating varieties of SPT is around 10%-15%. Between 1986 and 2006, 282 cystic tumors of the pancreas were observed. Among them a SPT was diagnosed in 8 patients (2.8%) with only one infiltrating variety. This was diagnosed in a 49-year-old female 13 years after the sonographic evidence of a small pancreatic cystic lesion interpreted as a pseudocyst. The tumor invaded a long segment of the portal-mesenteric vein confluence, and was removed with a total pancreatectomy, resection of the portal vein and reconstruction with the internal jugular vein. Histological examination confirmed the R-0 resection of the primary SPT, although a vascular invasion was demonstrated. The postoperative course was uneventful, but 32 mo after surgery the patient experienced diffuse liver metastases. Chemotherapy with different drugs was started. The patient is alive and symptom-free, with stable disease, 75 mo after surgery. Twenty-five patients with invasion of the portal vein and/or of mesenteric vessels were retrieved from the literature, 16 recent patients with tumor relapse after potentially curative resection were also retrieved. The best treatment remains a radical resection whenever possible, even in locally advanced or metastatic disease. The role of chemotherapy, and/or radiotherapy, is still to be defined.
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