| Abstract [eng] |
Introduction. Primary liver perivascular epithelioid cell tumor (PEComa) is an extremely rare entity. In this article, we present a case report with a systematic review of literature of patients diagnosed with primary liver PEComa. Together with an elaboration of diagnostic and treatment modalities. Materials and methods. A systematic search of literature was conducted using the terms: “perivascular epithelioid cell tumor” ,“PEComa”, “liver”, and “hepatic”. All the articles describing patients diagnosed with primary liver PEComa were included. Results. We identified a total of 136 patients with primary liver PEComa from 58 articles and a case from the present study with a significant preponderance of females (ratio 4:1) with a mean age of 45.9±11.9 years. Most of the patients (78 out of 136, 57.4 %) were asymptomatic. 106 patients (77.9 %) underwent surgical hepatic resection at the time of diagnosis, while 17 (12.5%) underwent surveillance. The recurrence and metastases were detected in 6 (4.4 %) and 5 (3.7 %) patients, respectively. Conclusions. Surgical resection remains the cornerstone of therapy. However, the presence of nonspecific imaging features makes difficult to reach the definite diagnosis preoperatively. Therefore, a multidisciplinary approach should be the gold standard in selecting treatment modality. Keywords. liver, perivascular epithelioid cell tumor, PEComa, systematic review. |
