2020 Volume 53 Issue 6 Pages 487-495
Perivascular epithelioid cell tumor (PEComa) is a family of rare mesenchymal tumors, derived from perivascular epithelioid cells. We report a case of laparoscopic hepatectomy for primary hepatic PEComa. A 45-year-old woman was referred to our hospital with a diagnosis of hepatocellular carcinoma because screening abdominal US revealed a hypervascular tumor in segment 2 of the liver. Laparoscopic partial hepatectomy was performed. The tumor was 2 cm in diameter and finally diagnosed as primary hepatic PEComa because it was immunohistochemically positive for both αSMA and HMB45. The background liver tissue showed moderate inflammation due to chronic hepatitis B virus infection but showed no fibrosis. The primary hepatic PEComa is extremely rare and criteria for malignancy have not yet been established. We believe this case is worth reporting because it is a rare pathology with difficulty in distinguishing from HCC.