Primary Pancreatic Perivascular Epithelioid Cell Tumor - Case Report and Prognostic Analysis

Velka Rosenfeld; Ella Benzaquen; Tsafrir Vanounou; Jean-Sebastien Pelletier

Volume : VI, Issue : XII, December - 2017

Primary Pancreatic Perivascular Epithelioid Cell Tumor - Case Report and Prognostic Analysis

Velka Rosenfeld, Ella Benzaquen, Tsafrir Vanounou, Jean Sebastien Pelletier

Abstract :

Perivascular epithelioid cell tumors (PEComas) are rare mesenchymal neoplasms and uncommonly arise from the pancreas. Our group encountered one such patient. Being that little is written about their behavior and prognosis, we sought to analyze the available literature and obtain a follow-up for previously reported patients to better ascertain the prognosis and biological behavior of these unusual neoplasms. Pancreatic PEComas predominantly occur in women with an average age of 50. Patients present with abdominal pain (8/17), GI bleeding (1/17), upper right quadrant bulge or pain (3/17), nonspecific symptoms (3/17) or asymptomatically (2/17). 11 cases met the inclusion criteria for the prognostic study. Two cases were excluded due to unavailable data and inability for follow-up. The prognosis of pancreatic PEComas is excellent, with a 5-year survival rate of 100% and recurrence rate of 22%. Pancreatic PEComas exhibit favorable survival rates but they do have the potential to recur. As such, we recommend radiological follow-up for all of these patients.