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Diagnostic Challenge of Primary Hepatic PEComa in a Young Woman with Prior Colorectal Cancer

Year: 2026

Type: article

Abstract: Primary hepatic perivascular epithelioid cell tumor (PEComa) is a rare mesenchymal neoplasm that can mimic common hepatic malignancies. A 28-year-old African woman with colorectal cancer in remission presented with 3 months of epigastric pain and tender hepatomegaly. Tumor markers were normal. Ultrasound and triphasic CT showed 2 large heterogeneous hypervascular hepatic masses with progressive enhancement and early venous drainage; hepatobiliary iminodiacetic acid scan (HIDA) SPECT-CT demonstrated photopenic lesions. Biopsy revealed epithelioid cells with focal HMB45, diffuse smooth muscle actin, and Ki-67 20%-30%, confirming hepatic PEComa. Resection was feasible but deferred; everolimus was started before loss to follow-up.

Source: ACG Case Reports Journal

DOI: 10.14309/crj.0000000000002377

Authors: Gerald Odong, Priyanka Panwar, Lavender Otom, Ivy Wakahora, Steven Idet, Sarah Kibe, Patricia Okiro, Saleem Abdulkarim, Ajua Alemanji, Amina Kidee, Samuel Gitau

Institutions: Aga Khan University Hospital Nairobi, Aga Khan University Nairobi

Language: English

Topic: Tuberous Sclerosis Complex Research

Subfield: Genetics (clinical)

Field: Medicine

Domain: Health Sciences

SDG: Good health and well-being

Open Access status: diamond

OpenAlex ID: W7225226120

FWCI:

Cites: 11

Cited by:

Related to: 10