Clinicopathological characteristics and surgical outcomes of primary hepatic perivascular epithelioid cell tumor (PEComa): A single-center cohort study.

BACKGROUND: Primary hepatic perivascular epithelioid cell tumor (PEComa) is a rare mesenchymal neoplasm with limited outcome data. This study evaluated clinicopathological features and long-term outcomes in a single-center cohort. METHODS: Forty-three patients with pathologically confirmed primary hepatic PEComa who underwent surgical resection at Seoul National University Hospital (2006-2024) were analyzed. Overall survival (OS), recurrence-free survival (RFS), and disease-specific survival (DSS) were estimated by Kaplan-Meier method. RESULTS: The cohort comprised 43 patients (72.1% women; median age 44.0 years (IQR 38.5-54.0)). R0 resection was achieved in 38 of 41 evaluable cases (92.7%). The median hospital stay was 7.0 days (IQR 6-8 days), and no major postoperative complications occurred (Clavien-Dindo grade ≥3). DSS was 100% throughout a median follow-up of 43.3 months; one intrahepatic recurrence and two deaths from unrelated malignancies occurred. The estimated 5-year OS and RFS were 95.8% (95% CI: 73.9-99.4%) and 93.6% (95% CI: 75.5-98.4%), respectively; because RFS incorporates these non-PEComa deaths, its lower confidence bound does not represent PEComa-related risk. CONCLUSIONS: Following surgical resection, disease-specific survival was 100%, and recurrence was uncommon; no death was attributable to PEComa. The small number of events, however, precluded assessment of prognostic factors; larger multicenter studies are needed.