Liver pecoma: perivascular epithelioid tumor. a case report.

Abstract

Background: Perivascular epithelioid cell tumors (PEComa) are an unusual mesenchymal tumors family, including the classic angiomyolipoma (AML), lypmphangioleiomyomatosis and clear-cell tumors, that can arise from different locations of the body [1–4]. We present a rare case of a Liver PEComa. Methods: A 58-year-old female with no background of cirrhosis or any other liver disease, who in an abdominal ultrasound, presented an exophytic heterogenic/solid mass of 5x6 cm in the left lateral section. Computed Tomography found a hypodense mass of 6.2x4.9x6.2 cm with fat density areas, quickly enhanced in the arterial phase and becoming less enhanced on venous and delayed phases of imaging, suggesting a PEComa as diagnosis. Laparoscopic Left Lateral Sectionectomy was performed. Pathology findings were a mix of three type of components: Adipose, vascular and perivascular epithelioid cells, HMB-45+, with conclusive findings of an angiomyolipoma. The patient was discharged at the second postoperative day, with no complications so far. Results: Liver PEComas were before considered as benign tumors, with surgical treatment in selected cases. The publications in the medical literature of some cases that confirm a malign transformation of AML have made the surgical indications for this pathology a controversial issue, recommending it in exophytic, >5cm or fast growing tumors [1–3]. Conclusion: Nowadays, liver PEComas are considerate as rare tumors with risk of malignancy in some cases, making the surgical intervention the primary treatment.