| Title |
Primary liver perivascular epithelioid cell tumor (PEComa): case report and literature review / |
| Authors |
Kvietkauskas, Mindaugas ; Samuolytė, Austėja ; Račkauskas, Rokas ; Lukšaitė-Lukštė, Raminta ; Karaliūtė, Gintarė ; Maskoliūnaitė, Vygantė ; Valkiūnienė, Rūta Barbora ; Sokolovas, Vitalijus ; Strupas, Kęstutis |
| DOI |
10.3390/medicina60030409 |
| Full Text |
|
| Is Part of |
Medicina.. Basel : MDPI AG. 2024, vol. 60, iss. 3, art. no. 409, p. [1-12].. ISSN 1010-660X. eISSN 1648-9144 |
| Keywords [eng] |
liver ; perivascular epithelioid cell tumor ; PEComa ; literature review |
| Abstract [eng] |
A primary liver perivascular epithelioid cell tumor (PEComa) is an extremely rare entity. In this article, we present a case report with a review of the literature on the patients diagnosed with primary liver PEComa and an elaboration of diagnostic and treatment modalities. A systematic literature search was conducted using the terms “perivascular epithelioid cell tumor”, “PEComa”, “liver”, and “hepatic”. All articles describing patients diagnosed with primary liver PEComa were included. We identified a total of 224 patients of primary liver PEComa from 75 articles and a case from the present study with a significant preponderance of females (ratio 4:1) and with a mean age of 45.3 ± 12.1 years. Most of the patients (114 out of 224, 50.9%) were asymptomatic. A total of 183 (81.3%) patients underwent surgical hepatic resection at the time of diagnosis, while 19 (8.4%) underwent surveillance. Recurrence and metastases were detected in seven (3.1%) and six (2.7%) patients, respectively. In conclusion, surgical resection remains the cornerstone of therapy; however, the presence of nonspecific imaging features makes it difficult to reach a definite diagnosis preoperatively. Therefore, a multidisciplinary approach should be the gold standard in selecting the treatment modality. |
| Published |
Basel : MDPI AG |
| Type |
Journal article |
| Language |
English |
| Publication date |
2024 |
| CC license |
|
