Published: 2021-05-28

Solid pseudopapillary epithelial neoplasia of the pancreas causing sinistral portal hypertension: a case report

Akshat Mishra, Girish D. Bakhshi, Rajesh Yadav, Samprati Doddamalappa, Mahesh Chanap, Sachin Sholapur, Sumit Malgaonkar, Kunal Suradkar


Solid pseudopapillary epithelial neoplasm (SPEN), also referred as Franz’s tumour or Hamoudi’s tumour is a low malignant potential epithelial neoplasm of the pancreas. It occurs at a much lower frequency than other cystic neoplasms of the pancreas. It occurs rarely and in young females. Although infrequently, large sized tumours are known to compress the splenic vein thereby resulting in extra hepatic portal venous obstruction (EHPVO) resulting in left sided portal hypertension (PHT). The resulting periportal collateral circulation poses an intraoperative challenge while approaching these tumors. Meticulous pre-operative planning can go a long way in managing such a case and providing complete surgical cure. We present a case of SPEN in a 37 years old female which was discovered incidentally and managed surgically to achieve a complete cure to both the tumour and the left sided PHT.


SPEN, Left sided portal hypertension

Full Text:



Santini D, Poli F, Lega S. Solid-papillary tumors of the pancreas: histopathology. JOP. 2006;7:131-6.

Sandlas G, Tiwari C. Solid Pseudopapillary Tumor of Pancreas: A Case Report and Review of Literature. Indian J Med Paediatr Oncol. 2017;38:207-9.

Chakhachiro ZI, Zaatari G. Solid-pseudopapillary neoplasm: A pancreatic enigma. Arch Pathol Lab Med. 2009.

Papavramidis T, Papavramidis S. Solid pseudopapillary tumors of the pancreas: review of 718 patients reported in English literature. J Am Coll Surg. 2005;200:965-72.

Klimstra DS, Wenig BM, Heffess CS. Solid-pseudopapillary tumor of the pancreas: a typically cystic carcinoma of low malignant potential. Semin Diagn Pathol. 2000;17:66-80

Panieri E, Krige JEJ, Bornman PC, Graham SM, Terblanche J, Cruse J. Operative management of papillary cystic neoplasms of the pancreas. J Am Coll Surg. 1998;186:319-24.

Nishimori I, Kohsaki T, Tochika X. Non-cystic solidpseudopapillary tumor of the pancreas showing nuclear accumulation and activating gene mutation of beta-catenin. Pathol Int. 2006;56;707-11.

Kucera JN, Kucera S, Perrin SD, Caracciolo JT, Schmulewitz N, Kedar RP. Cystic lesions of the pancreas: Radiologic-endosonographic correlation. Radiographics. 2012.

Cantisani V, Mortele KJ, Levy A. MR Imaging features of solid pseudopapillary tumor of the pancreas in adult and pediatric patients. Am J Roentgenol. 2003;181:395-401.

Kalb B, Sarmiento JM, Kooby DA, Adsay NV, Martin DR. MR imaging of cystic lesions of the pancreas. RadioGraphics 2009;29:1749-65.

Lee DH, Yi BH, Lim JW, Ko YT. Sonographic findings of solid and papillary epithelial neoplasm of the pancreas. J Ultrasound Med. 2001;20:1229-32.

Wani NA, Lone TK, Shah AI, Khan AQ, Malik RA. Malignant solid pseudopapillary tumor of pancreas causing sinistral portal hypertension. Indian J Pathol Microbiol. 2011;54:152-5.

Reddy A, Sanniyasi S, George DJ, Narayanan CD. A rare case report of Solid Pseudopapillary Tumor of the pancreas with portal hypertension. Int J Surg Case Rep. 2016;22:35-8.

Aikot S, Manappallil RG, Pokkattil S, Kakkattil A. Solid pseudopapillary neoplasm of pancreas: an unusual aetiology for haematochezia. BMJ Case Rep. 2018;225332.

Nakamura S, Takayama Y, Kuboki Y, Haruyama H, Kishino M, Konishi H, Hatori T, Furukawa T, Shiratori K. A case of solid pseudopapillary neoplasm of the pancreas presenting with left-sided extrahepatic portal hypertension. Intern Med. 2010;49(16):1749-53.

Moyana TN, Macdonald DB, Martel G, Pyatibrat S, Lee G, Capitano M. Pancreatic Neuroendocrine Tumors Complicated by Sinistral Portal Hypertension: Insights into Pathogenesis. J Pancreat Cancer. 2017;3(1):71-77.

Yagcı A, Yakan S, Coskun A, Erkan N, Yıldırım M, Yalcın E, Postacı H. Diagnosis and treatment of solid pseudopapillary tumor of the pancreas: experience of one single institution from Turkey. World J Surg Oncol. 2013;11:308.

Tang LH, Aydin H, Brennan MF, Klimstra DS. Clinically aggressive solid pseudopapillary tumor of the pancreas. A report of two cases with components of undifferentiated carcinoma and a comparative clinicopathologic analysis of 34 conventional cases. Am J Surg Pathol. 2005;29:512-9.

Matsunou H, Konishi F. Papillary-cystic neoplasm of the pancreas: a clinicopathologic study concerning the tumor aging and malignancy of nine cases Cancer. 1990;65:283-91.