Background: Solid pseudopapillary epithelial neoplasm of pancreas (SPEN) also known as papillary epithelial neoplasm and cystic-solid papillary carcinoma of the pancreas is an uncommon neoplasm of exocrine pancreas. It predominantly occurs in young women in their twenties and thirties and accounts for 1-2% of all pancreatic tumors. It is a peculiar tumor for having low grade malignant potential and an excellent prognosis. Most patients are asymptomatic at diagnosis and may present with gradually enlarging abdominal mass or vague abdominal pain. The diagnosis is usually done by imaging (CECT, MRI) and histopathology. Here we report a case of 22 years old pregnant woman who was referred to our hospital with a history of gradually increasing abdominal lump and vague abdominal pain since 1 year. She was diagnosed with SPN by imaging and histopathology.

Case Report: A 22 years old female with full term gestation was referred to us in view of gradually increasing abdominal mass and history of vague abdominal pain since 1 year. At 12 weeks of gestation she was investigated at her native place and was diagnosed with a mass in left renal fossa. In our hospital she delivered male child at full term by normal vaginal delivery. In postnatal period CECT was done which showed a lobulated lesion with internal calcification in left anterior para-renal space. A USG guided trucut biopsy confirmed the diagnosis of pseudopapillary epithelial neoplasm of pancreas. Distal pancreaticosplenectomy with left radical nephrectomy and left hemicolectomy was done. At the time of discharge patient was advised regular follow up. We are reporting this case because SPN are uncommon tumors and their co-occurrence with pregnancy is even rarer.

Conclusion: SPENs are rare but curable neoplasm of exocrine pancreas affecting young females in their 2nd or 3rd decade. The diagnosis is sometimes difficult because of a wide differential diagnosis however once diagnosed it has an excellent prognosis because of its low malignant potential. Presence of gradually increasing, well defined, cystic pancreatic mass in young females should raise the suspicion of SPN.

Keywords: Pseudopapillary epithelial neoplasm of pancreas, Pregnancy, Imaging, Surgical resection.

1. Frost M, Krige JE, Bornman PC, Panieri E, Beningfield SJ, Wainwright H. Solid pseudopapillary epithelial neoplasm--a rare but curable pancreatic tumour inyoung women. S Afr J Surg. 2011 Apr;49(2):75-6, 78-81.
2. Frantz VK. Tumors of the pancreas.In: Atlas of tumor pathology, section VII, fascicles 27 and 28. Washington, DC: US Armed Forces Institute of Pathology; 1959
3. Papavramidis T, Papavramidis S. Solid pseudopapillary tumours of the pancreas: review of 718 patients reported in english literature.J Am Coll Surg 2005;200:965–72
4. Patil TB, Shrikhande SV, Kanhere HA, Saoji RR, Ramadwar MR, Shukla PJ. Solid pseudopapillary neoplasm of the pancreas: a single institution experience of 14 cases. HPB : The Official Journal of the International Hepato Pancreato Biliary Association. 2006;8(2):148-150.
5. Sunkara S, Williams TR, Myers DT, Kryvenko ON. Solid pseudopapillary tumours of the pancreas: spectrum of imaging findings with histopathological correlation. The British Journal of Radiology. 2012;85(1019):e1140-e1144.
6. Liu B-A, Li Z-M, Su Z-S, She X-L. Pathological differential diagnosis of solid-pseudopapillary neoplasm and endocrine tumors of the pancreas. World Journal of Gastroenterology : WJG. 2010;16(8):1025-1030.
7. Friedman AC, Lichtenstein JE, Fishman EK, Oertel JE, Dachman AH, Siegelman SS.Solid and papillary epithelial neoplasm of the pancreas. Radiology. 1985 Feb;154(2):333-7.
8. Guo N, Zhou QB, Chen RF, et al. Diagnosis and surgical treatment of solid pseudopapillary neoplasm of the pancreas: analysis of 24 cases. Canadian Journal of Surgery. 2011;54(6):368-374.
9. Boyd CA, Benarroch-Gampel J, Kilic G, Kruse EJ, Weber SM, Riall TS. Pancreatic Neoplasms in Pregnancy: Diagnosis, Complications, and Management. Journal of Gastrointestinal Surgery. 2012;16(5): 1064-1071.
10. Ganepola GA, Gritsman AY, Asimakopulos N, Yiengpruksawan A. Are pancreatic tumors hormone dependent?: A case report of unusual, rapidly growing pancreatic tumor during pregnancy, its possible relationship to female sex hormones, and review of the literature. Am Surg. 1999;65:105–11. 
11. Lakhtakia R, Al-Wahaibi K, Zahid KF, Malik KA, Burney IA. Solid Pseudopapillary Neoplasm of the Pancreas: A Case Report with Review of the Diagnostic Dilemmas and Tumor Behavior. Oman Medical Journal. 2013;28(6):441-444.
12. Santini D, Poli F, Lega S; Solid-papillary tumors of the pancreas: histopatology. JOP, 2006; 7(1): 131- 136.
13. Papavramidis T, Papavramidis S; Solid pseudopapillary tumors of the pancreas: review of 718 patients reported in English literature. J Am Coll Surg., 2005; 200(6): 965–972
14. Khaniya S, Shakya VC, Koirala R. Solid pseudopapillary tumor in an ectopic pancreas: an unusual presentation. Journal of Surgical Case Reports. 2017;2017(3):rjx050.
15. Vassos N, Agaimy A, Klein P, Hohenberger W, Croner RS. Solid-pseudopapillary neoplasm (SPN) of the pancreas: case series and literature review on an enigmatic entity. International Journal of Clinical and Experimental Pathology. 2013;6(6):1051-1059.
16. Chaudhary V, Bano S. Imaging of the pancreas: Recent advances. Indian Journal of Endocrinology and Metabolism. 2011;15(Suppl1):S25-S32. doi:10.4103/2230-8210.83060.
17. Kim MJ, Jang SJ, Yu E. Loss of E-cadherin and cytoplasmic-nuclear expression of beta-catenin are the most useful immunoprofiles in the diagnosis of solid-pseudopapillary neoplasm of the pancreas. Hum Pathol. 2008 Feb;39(2):251-8.
18. Afridi SA, Kazaryan AM, Marangos IP, et al. Laparoscopic Surgery for Solid Pseudopapillary Tumor of the Pancreas. JSLS : Journal of the Society of Laparoendoscopic Surgeons. 2014;18 (2):236-242.
19. Boyd CA, Benarroch-Gampel J, Kilic G, Kruse EJ, Weber SM, Riall TS. Pancreatic Neoplasms in Pregnancy: Diagnosis, Complications, and Management. Journal of Gastrointestinal Surgery. 2012;16(5): 1064-1071.
20. Wiseman JE, Yamamoto M, Nguyen TD, Bonadio J, Imagawa DK. Cystic pancreatic neoplasm in pregnancy: a case report and review of the literature. Arch Surg. 2008 Jan;143(1):84-6.

Dr Muskaan Chhabra

Department of Obstetrics and Gynaecology,

Grant Govt. Medical College & Sir JJ Group of Hospitals Mumbai