Primary Hepatic Perivascular Epithelioid Tumor (PEComa) With High Risk of Malignancy: A Case Report

TL;DR

A rare case of primary liver PEComa with high malignancy risk is reported, highlighting the need for accurate diagnosis and better management strategies.

Contribution

This case report presents a rare primary hepatic PEComa with confirmed malignant behavior.

Findings

The patient required a second surgery due to positive surgical margins after initial partial hepatectomy.

Immunohistochemical studies confirmed PEComa with coexpression of melanocytic and muscle markers.

The case highlights the lack of validated risk stratification for hepatic PEComas.

Abstract

Perivascular Epithelioid Tumors (PEComas) are predominantly present in uterine or gastric tissues. Liver presentations are uncommon and primary hepatic presentations are extremely rare. This is the case of a 31-year-old female patient, with previous diagnosis of hepatic abscess, the patient presented with a one-month history of abdominal pain and bilious emesis. Abdominal MR reported a lesion suggestive of hepatic adenoma. The team performed a partial hepatectomy, and histopathologic and immunohistochemical (IHC) studies reported a PEComa with high risk of confirmed malignant behavior metastatic workup. A complete left laparoscopic hepatectomy was performed due to positive surgical margins. Careful complete histologic and IHC studies are required for diagnosis. IHC reveals coexpression of melanocytic and muscle markers. These studies are usually performed after hepatectomy, the leading management strategy. Long-term follow-up and validated risk stratification scales for hepatic PEComas are needed. This report underscores the critical role of accurate diagnosis and the absence of precise information for management and prognosis of hepatic PEComas.

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