Solid pseudopapillary neoplasm of the pancreas: report of a rare case and review of the literature

Arzu Nese Yener,Manuk Manukyan,Rahmi Cubuk,Ahmet Midi

doi:10.5146/tjpath.2013.01192

Abstract

Solid pseudopapillary neoplasm, a rare primary neoplasm of the pancreas that typically affects young women, is a relatively indolent entity with favorable prognosis. We here report a 20-year-old young girl with solid pseudopapillary neoplasm who presented with mild dull abdominal discomfort without any significant laboratory findings. On MRI, a heterogenous mass was found at the distal pancreas. The patient underwent en-block distal pancreatectomy with splenectomy with the presumptive diagnosis of cystic neoplasm of the pancreas. The tumor was well-circumscribed, encapsulated, 5.5 cm in the greatest dimension and showed typical papillary and pseudopapillary structures. Capsular invasion was seen on focal areas. The patient was not given any adjuvant therapy and shows no sign of disease after six months follow-up. It is important to differentiate this tumor from other pancreatic neoplasms because this neoplasm is amenable to cure after complete surgical resection even in cases with capsular invasion, unlike malignant tumors of the pancreas.

Highlights

Solid pseudopapillary neoplasm (SPN) of the pancreas almost exclusively affects young women and is a rare primary neoplasm (1-4)
We aimed to present the case of a 20-year-old young girl with an SPN located in the distal part of the pancreas and briefly review the literature on this rare entity
SPN was first described by Gruber Frantz in 1959 and many cases or case series have been reported so far (27)

Summary

INTRODUCTION

Solid pseudopapillary neoplasm (SPN) of the pancreas almost exclusively affects young women and is a rare primary neoplasm (1-4). We aimed to present the case of a 20-year-old young girl with an SPN located in the distal part of the pancreas and briefly review the literature on this rare entity. Computerized tomography and magnetic resonance imaging revealed a well-circumscribed, partly cystic and partly solid mass measuring 57x48 mm in the tail of the pancreas (Figure 1A,B). We observed a tumor mainly composed of cystic cavities lined by friable tissue and cholesterol clefts (Figure 3A). The solid parts grossly described were showing diffuse growth pattern with minimal supporting fibrovascular stroma They were composed of papillary, pseudopapillary and microcystic structures with dyscohesive neoplastic cells (Figure 3B). The tumor was separated from the normal pancreatic tissue with a thick fibrous capsule in which there was infiltration of the tumor cells in some areas (Figure 3D). The patient did not undergo any adjuvant therapy and has been doing well in the year after her operation

DISCUSSION

B Figure 1

C Figure 4