Hepatic PEComas ‒ A national multicenter study. Clinical, pathological, and surgical outcomes

PEComas are neoplasms originating from perivascular epithelioid cells. Their incidence is higher in patients with tuberous sclerosis; however, hepatic PEComas are exceptionally rare compared to those in other locations. Diagnostic confirmation relies on histopathological findings and immunohistochemical phenotypes.

To evaluate the clinical-radiological presentation, histopathological characteristics, and surgical outcomes of a multicenter cohort of hepatic PEComas.

A retrospective, multicenter, nationwide observational study of patients with histologically confirmed hepatic PEComa who underwent surgery at Spanish centers between 2010 and 2024.

Twenty-two patients were included; 21 (95.5%) were women, with a mean age of 54.7 years. Mean tumor size was 4.3 cm. Preoperative radiological diagnosis was challenging, with PEComa identified in only one case (4.5%). Preoperative biopsy was performed in 8 patients, yielding a diagnosis of PEComa in 6 cases (75%). Surgical procedures consisted of minor hepatectomy in 14 patients (63.6%) and major hepatectomy in 8 patients (36.4%). The 90-day mortality rate was 0%. Mean hospital stay was 4.5 days. Immunohistochemical analysis was positive for: HMB45 (19 patients, 90.5%), Melan-A (17, 81%), and actin (13, 61.9%). After a median follow-up of 49 months, no recurrences were detected.

Preoperative diagnosis of PEComa poses a radiological challenge, frequently requiring biopsy for confirmation. Surgical treatment was not associated with mortality, showed a relevant complication rate of 18.2%, and no recurrences were observed.