Primary hepatic PEComa: A case report

Abstract

Author(s): Kelly Carter*

Mesenchymal neoplasms of the liver are very uncommon. As mesenchymal neoplasms, hepatic PEComas are part of the broader spectrum of rare liver tumors that arise from non-epithelial cell origins. Understanding the unique characteristics and clinical behavior of these tumors is crucial for early and accurate diagnosis, leading to appropriate treatment and improved patient outcomes. Herein, we present the case of a patient who underwent right hemihepatectomy with segment (IVA) preservation for a tumor identified as Fibrolamellar Hepatocellular Carcinoma (FL-HCC) by imaging investigations. The final histopathological examination revealed a Benign Epithelioid tumor (PEComa), with resection representing the standard of care. Clinical suspicion, the use of multiple imaging modalities, and a multidisciplinary approach are essential to avoid misdiagnosing unusual tumours such as hepatic PEComa. Additionally, long-term follow-up is crucial owing to the unpredictable natural history of PEComas.

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