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

Cardosa, MF; Ferreira de Lima, S; Knoblich, M; Afonso, I; Mendes, C

http://hdl.handle.net/10400.17/4239

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Abstract(s)

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 broad but the immunohistochemical biomarkers establish the diagnosis. A 4-year-old girl was referred to a pediatric gastroenterology clinic 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 by ultrasound, and the magnetic resonance imaging revealed a highly vascularized intraperitoneal tumor with well-defined limits. Surgical mass resection was performed, and the histology revealed morphological and immunohistochemical aspects of a perivascular epithelioid cell tumor of the abdominal wall. Given the size of the lesion (> 5 cm) and admitting uncertain malignant potential, she was submitted to margin enlargement. Four years later, she remains asymptomatic without clinical or imagiological evidence of relapse.

Keywords

Incidental Findings Perivascular Epithelioid Cell Neoplasms/diagnosis Perivascular Epithelioid Cell Neoplasms/surgery Abdominal Wall Treatment Outcome Case Report HDE GAS PED HDE CIR PED