Tumor de células epitelioides perivasculares (PECOMA) hepático: relato de caso

Abstract

We report a case of hepatic perivascular epithelioid cell tumor (PECOMA) diagnosed in a female patient at Walter Cantídio University Hospital. Initially, a medical record was reviewed and then a bibliographic search of the national and international literature was obtained, obtained from the databases: SciELO and MEDLINE. PEComas are rare tumors, characterized by the coexpression of melanocytic and muscular transcription factors. The case of a 49-year-old woman with recurrent bouts of pain and epigastric discomfort after feeding. Computed tomography of the abdomen revealed a large, predominantly hypodense, heterogeneous hepatic mass with regular contours and defined limits, showing areas of necrosis and internal calypsoar arteries, including radial and aneurysmal dilatations. Patient was submitted to partial hepatectomy. Macroscopically, the hepatic segments showed a well-defined tumor, measuring 15.0 cm, brownish-brown, with a necrotic appearance. Under microscopy, the tumor was composed of polygonal epithelioid cells, positive for melanocytic markers to immunohistochemistry. Conclusions: PE tumors are rare tumors, which may arise in several regions and are categorized as benign, with uncertain or malignant potential. We report the case of a patient who, after excision of the lesion, developed a clinically stable condition without recurrence after six months of follow-up.