[Misdiagnosis of a giant hepatic perivascular epithelioid cell tumor: A case report].

Perivascular epithelioid cell tumor (PEComa) is a rare mesenchymal neoplasm, and giant (maximum diameter is ≥10 cm) hepatic PEComa is even more uncommon. We report a case of giant hepatic PEComa that was preoperatively misdiagnosed as fibrolamellar hepatocellular carcinoma. The patient was a middle-aged woman who presented with upper abdominal discomfort and was admitted to Xiangya Hospital of Central South University on August 21, 2025, for evaluation of a hepatic space-occupying lesion. She had no history of chronic liver disease, and relevant laboratory tests showed no significant abnormalities. Multiple preoperative imaging modalities revealed a giant hepatic mass, which was initially diagnosed as fibrolamellar hepatocellular carcinoma. The tumor was completely resected (the size of mass was 14.3 cm×13.5 cm×10.0 cm) and the final diagnosis of hepatic PEComa was established by histopathological examination and immunohistochemical analysis. During a 6-month postoperative follow-up period, the patient remained asymptomatic and showed no evidence of recurrence. The diagnosis of hepatic PEComa is challenging because of its lack of specific clinical manifestations and characteristic imaging findings. Through retrospective analysis of this case, the causes of misdiagnosis were explored and the key points for differential diagnosis were summarized. This report aims to improve awareness of hepatic PEComa among clinicians and imaging specialists, provide additional references for preoperative imaging diagnosis, reduce the rate of misdiagnosis, optimize treatment decision-making, and ultimately improve patient outcomes.