Hepatic PEComa: A rare mesenchymal tumor with epithelioid features. A case report and review of the literature.
We present a case of a hepatic PEComa in a 43-year-old man with no comorbidities and otherwise good health, who presented with abdominal discomfort and right upper quadrant tenderness. Laboratory findings were unremarkable. The patient underwent robotic-assisted left hemihepatectomy, resulting in negative margins. This case illustrates the diagnostic challenges of hepatic PEComas, given their rarity and morphologic overlap with primary liver tumors. It emphasizes the necessity of integrating histopathology, immunohistochemistry, and molecular testing for accurate diagnosis, thereby guiding appropriate clinical management and avoiding misclassification.