Very Rare Liver Tumor: PEComa Case Report with and a Review of Literature

Abstract Perivascular epithelioid cell tumors (PEComas) are very rare mesenchymal tumors. In addition to being rare in general, they are even rarer in the liver. There are various subtypes and there is only one case of liver PEComa reported in the clear cell type in the literature to date. We present the second clear cell type liver PEComa in this case by reviewing the literature data. The hypodense, smooth-edged, ovoid lesion was detected on computed tomography (CT) for performing abdominal pain in a 41-year-old female patient. Magnetic resonance imaging (MRI) was then performed for lesion characterization. The lesion was hypointense on T1-weighted imaging (T1WI), hyperintense on T2-weighted imaging (T2WI). In dynamic phases, it showed marked enhancement on the arterial phase and capsular enhancement with central washout on the portal and late venous phases. The posterior branch of the right portal vein extended into the mass. The lesion was excised and the pathological result was epithelioid clear cell subtype of PEComa. Although the imaging findings are generally nonspecific and certain diagnosis is made histopathologically, radiologists should consider PEComa in the differential diagnosis in the presence of intensely enhanced lesion on the right lobe in female patients. Also, the “large vessel sign” may help in the diagnosis.