Perivascular Epithelioid Cell Tumor: A Case Report of a Rare Entity

Date of submission: 23-04-2020 | Date of acceptance: 12-10-2020 | Published: 03-01-2021

Abstract

Perivascular epithelioid cell tumor is a rare mesenchymal tumor with myomelanocytic differentiation. It mainly occurs in middle-aged females and can be found at any location. The differential diagnosis is fairly broad but the immunohistochemical biomarkers establish the diagnosis. Afour-year-old girl was referred to pediatric gastroenterology consultation due to recurrent umbilical pain and the workup revealed biliary lithiasis. She remained in follow-up, asymptomatic, no physical findings and stable ultrasound. Six years later, a supra-umbilical mass was detected on ultrasound, and the magnetic resonance imaging revealed an intra-peritoneal tumor with well-defined limitsand highly vascularized. Surgical mass resection was performed, and histology revealed morphological and immunohistochemical aspects of a Perivascular epithelioid cell tumorof the abdominal wall. Given the size of the lesion (> 5 cm) and admitting uncertain malignant potential, she was submitted to margin enlargement. Three years later, she remains asymptomatic without clinical or imagiological evidence of relapse.