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

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.