DOI: 10.14309/crj.0000000000002377 ISSN: 2326-3253
Diagnostic Challenge of Primary Hepatic PEComa in a Young Woman with Prior Colorectal Cancer
Gerald Odong, Priyanka Panwar, Lavender Otom, Ivy Wakahora, Steven Idet, Sarah Kibe, Patricia Okiro, Saleem Abdulkarim, Ajua Alemanji, Amina Kidee, Samuel GitauABSTRACT
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.