JOURNAL OF CLINICAL AND BIOMEDICAL SCIENCES

Article

Journal of Clinical and Biomedical Sciences

Year: 2026, Volume: 16, Issue: 1, Pages: 75- 80

Case Report

Pediatric Solid Pseudopapillary Tumor of the Pancreas: Case Series and Literature Review

Received Date:15 August 2025, Accepted Date:16 December 2025, Published Date:21 April 2026

Abstract

We describe a case series of children with solid pseudopapillary tumor of the pancreas and present a literature review from a pediatric perspective. The tumor occurs overwhelmingly in young women, is rare, often large and has a low-grade malignant potential. Most patients present with non-specific abdominal symptoms, although occasionally it may be discovered incidentally in asymptomatic individuals. The diagnosis is based on characteristic findings seen in imaging modalities and confirmatory histopathology and immunohistochemical profiles. Treatment is surgical and the operative approach can include enucleation, segmental pancreatectomy or Whipple procedure, depending on the tumor’s pancreatic anatomic location. Overall, with complete resection, the neoplasm is associated with an excellent prognosis, with outcomes more favorable in children than in adults.

Keywords: Pancreatic tumor, Children, Abdominal pain, Cytology, Pancreatectomy

References

1. Frantz VK. Tumors of the Pancreas in Atlas of Tumor Pathology. Section VII. Fascicles 27 and 28. Armed Forces Institute of Pathology. Washington DC. 1959. Pages 32-5.

2. American Cancer Society. Cancer Statistics Center. Retrieved May 28, 2025. Available from: https://cancerstatisticscenter.org.

3. Colak MA, Joshi S, Freeman AJ, Gariepy CE, Rasmussen SK, Nathan JD. Incidence, Management, and Survival of Pancreatic Malignancies in Children: A Population-Based SEER Study. Journal of Pediatric Surgery. 2025; 60 (4). Available from: https://doi.org/10.1016/j.jpedsurg.2025.162197

4. Li Q, Wei Q, Dai Y. Epidemiology, pathology, and physiology of pancreatic diseases. Integrative Pancreatic Intervention Therapy. 2021; Available from: https://doi.org/10.1016/b978-0-12-819402-7.00002-4

5. Papavramidis T, Papavramidis S. Solid Pseudopapillary Tumors of the Pancreas: Review of 718 Patients Reported in English Literature. Journal of the American College of Surgeons. 2005; 200 (6). Available from: https://doi.org/10.1016/j.jamcollsurg.2005.02.011

6. Speer AL, Barthel ER, Patel MM, Grikscheit TC. Solid pseudopapillary tumor of the pancreas: a single-institution 20-year series of pediatric patients. Journal of Pediatric Surgery. 2012; 47 (6). Available from: https://doi.org/10.1016/j.jpedsurg.2012.03.026

7. Kosmahl M, Seada LS, Jänig U, Harms D, Klöppel G. Solid-pseudopapillary tumor of the pancreas: its origin revisited. Virchows Archiv. 2000; 436 (5). Available from: https://doi.org/10.1007/s004280050475

8. Rodriguez-Matta E, Hemmerich A, Starr J, Mody K, Severson EA, Colon-Otero G. Molecular genetic changes in solid pseudopapillary neoplasms (SPN) of the pancreas. Acta Oncologica. 2020; 59 (9). Available from: https://doi.org/10.1080/0284186x.2020.1792549

9. Abraham SC, Klimstra DS, Wilentz RE, Yeo CJ, Conlon K, Brennan M, et alSolid-Pseudopapillary Tumors of the Pancreas Are Genetically Distinct from Pancreatic Ductal Adenocarcinomas and Almost Always Harbor β-catenin Mutations. The American Journal of Pathology. 2002; 160 (4). Available from: https://doi.org/10.1016/s0002-9440(10)62563-1

10. Wang BG, Mani H, Wang ZQ, Li W. Cytological Diagnosis of Pancreatic Solid-Pseudopapillary Neoplasm: A Single-Institution Community Practice Experience. Diagnostics. 2022; 12 (2). Available from: https://doi.org/10.3390/diagnostics12020449

11. Choi SH, Kim SM, Oh JT, Park JY, Seo JM, Lee SK. Solid pseudopapillary tumor of the pancreas: a multicenter study of 23 pediatric cases. Journal of Pediatric Surgery. 2006; 41 (12). Available from: https://doi.org/10.1016/j.jpedsurg.2006.08.024

12. Kovac JD, Djikic-Rom A, Bogdanovic A, Jankovic A, Grubor N, Djuricic G et alThe Role of MRI in the Diagnosis of Solid Pseudopapillary Neoplasm of the Pancreas and Its Mimickers: A Case-Based Review with Emphasis on Differential Diagnosis. Diagnostics. 2023; 13 (6). Available from: https://doi.org/10.3390/diagnostics13061074

13. Law JK, Ahmed A, Singh VK, Akshintala VS, Olson MT, Raman SP, et alA Systematic Review of Solid-Pseudopapillary Neoplasms. Pancreas. 2014; 43 (3). Available from: https://doi.org/10.1097/mpa.0000000000000061

14. Lee SE, Jang J, Hwang DW, Park KW, Kim SW. Clinical Features and Outcome of Solid Pseudopapillary Neoplasm. Archives of Surgery. 2008; 143 (12). Available from: https://doi.org/10.1001/archsurg.143.12.1218

15. Mao C, Guvendi M, Domenico DR, Lim K, Thomford NR, Howard JM. Papillary cystic and solid tumors of the pancreas: A pancreatic embryonic tumor? Studies of three cases and cumulative review of the world's literature. Surgery. 1995; 118 (5). Available from: https://doi.org/10.1016/s0039-6060(05)80271-5

16. Horisawa M, Niinomi N, Sato T, Yokoi S, Oda K, Ichikawa M, et alFrantz's tumor (solid and cystic tumor of the pancreas) with liver metastasis: Successful treatment and long-term follow-up. Journal of Pediatric Surgery. 1995; 30 (5). Available from: https://doi.org/10.1016/0022-3468(95)90701-7

17. Omiyale AO. Solid pseudopapillary neoplasm of the pancreas. World Journal of Hepatology. 2021; 13 (8). Available from: https://doi.org/10.4254/wjh.v13.i8.896

18. Qiu L, Trout AT, Ayyala RS, Szabo S, Nathan JD, Geller JI, et alPancreatic Masses in Children and Young Adults: Multimodality Review with Pathologic Correlation. RadioGraphics. 2021; 41 (6). Available from: https://doi.org/10.1148/rg.2021210008

19. Singhi AD, Lilo M, Hruban RH, Cressman KL, Fuhrer K, Seethala RR. Overexpression of Lymphoid Enhancer-Binding Factor 1 (LEF1) in solid-pseudopapillary neoplasms of the pancreas. Modern Pathology. 2014; 27 (10). Available from: https://doi.org/10.1038/modpathol.2014.40

20. Liu BA, Li ZM, Su ZS, She XL. Pathological differential diagnosis of solid-pseudopapillary neoplasm and endocrine tumors of the pancreas. World Journal of Gastroenterology. 2010; 16 (8). Available from: https://doi.org/10.3748/wjg.v16.i8.1025

21. Tang LH, Aydin H, Brennan MF, Klimstra DS. Clinically Aggressive Solid Pseudopapillary Tumors of the Pancreas. American Journal of Surgical Pathology. 2005; 29 (4). Available from: https://doi.org/10.1097/01.pas.0000155159.28530.88

22. Campanile M, Nicolas A, LeBel S, Delarue A, Guys JM, de Lagausie P. Frantz's tumor: Is mutilating surgery always justified in young patients?. Surgical Oncology. 2011; 20 (2). Available from: https://doi.org/10.1016/j.suronc.2009.12.003

23. Waters AM, Russell RT, Maizlin II, Beierle EA, CCDR Group. Comparison of Pediatric and Adult Solid Pseudopapillary Neoplasms of the Pancreas. Journal of Surgical Research. 2019; 242 Available from: https://doi.org/10.1016/j.jss.2019.04.070

24. Choi SH, Kim SM, Oh JT, Park JY, Seo JM, Lee SK. Solid pseudopapillary tumor of the pancreas: a multicenter study of 23 pediatric cases. Journal of Pediatric Surgery. 2006; 41 (12). Available from: https://doi.org/10.1016/j.jpedsurg.2006.08.024

25. Lee CW, Namgoong JM, Kim DY, Kim SC, Lee SY, Cho Y, et alPerioperative Outcomes and Surgical Indications of Minimally Invasive Pancreatectomy for Solid Pseudopapillary Tumor in Pediatric Patients. Advances in Pediatric Surgery. 2018; 24 (2). Available from: https://doi.org/10.13029/aps.2018.24.2.76

26. Kumar NAN, Bhandare MS, Chaudhari V, Sasi SP, Shrikhande SV. Analysis of 50 cases of solid pseudopapillary tumor of pancreas: Aggressive surgical resection provides excellent outcomes. European Journal of Surgical Oncology. 2019; 45 (2). Available from: https://doi.org/10.1016/j.ejso.2018.08.027

27. Yu PF, Hu ZH, Wang XB, Guo JM, Cheng XD, Zhang YL, et alSolid pseudopapillary tumor of the pancreas: A review of 553 cases in Chinese literature. World Journal of Gastroenterology. 2010; 16 (10). Available from: https://doi.org/10.3748/wjg.v16.i10.1209

28. Reddy SHS, Zen Y, Aluvihare V, Menon KV. Liver Transplantation for Metastases From Solid Pseudopapillary Tumor of the Pancreas: A Case Report and Review of Literature. Transplantation Direct. 2022; 8 (6). Available from: https://doi.org/10.1097/txd.0000000000001328

29. Sumida W, Kaneko K, Tainaka T, Ono Y, Kiuchi T, Ando H. Liver transplantation for multiple liver metastases from solid pseudopapillary tumor of the pancreas. Journal of Pediatric Surgery. 2007; 42 (12). Available from: https://doi.org/10.1016/j.jpedsurg.2007.08.056

30. Bender AM, Thompson ED, Hackam DJ, Cameron JL. Solid Pseudopapillary Neoplasm of the Pancreas in a Young Pediatric Patient. Pancreas. 2018; 47 (10). Available from: https://doi.org/10.1097/mpa.0000000000001183

31. Abudalou M, Vega EA, Dhingra R, Holzwanger E, Krishnan S, Kondratiev S, et alSolid pseudopapillary neoplasm-diagnostic approach and post-surgical follow up: Three case reports and review of literature. World Journal of Clinical Cases. 2021; 9 (7). Available from: https://doi.org/10.12998/wjcc.v9.i7.1682

Copyright

This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

Published By Sri Devaraj Urs Academy of Higher Education, Kolar, Karnataka

DON'T MISS OUT!

Subscribe now for latest articles and news.