CASI CLINICI   

Chirurgia 2015 February;28(1):15-8

Copyright © 2015 EDIZIONI MINERVA MEDICA

lingua: Inglese

Malignant PEComa of the liver

Massani M. ¹, Stecca T. ^{1, 2}, Cugini C. ³, Ruffolo C. ¹, Bonariol L. ¹, Pauletti B. ^{1, 2}, Canal F. ⁴, Fabris L. ^{2, 5, 6}, Morana G. ³, Bassi N. ^{1, 2}

¹ Fourth Surgical Emergency and HPB Unit, 2nd Department of Surgery, Treviso Regional Hospital, Treviso, Italy; ² Department of Surgery, Oncology and Gastroenterology, University of Padua, Padua, Italy; ³ Department of Radiology, Treviso Regional Hospital, Treviso, Italy; ⁴ Pathological Anatomy Department, Treviso Regional Hospital, Treviso, Italy; ⁵ Center for Liver Research (CeLiveR), Ospedali Riuniti di Bergamo, Bergamo, Italy; ⁶ Department of Surgery and Interdisciplinary Medicine, University of Milano‑Bicocca, Milan, Italy

Perivascular epithelioid cell tumours (PEComa) are described as “mesenchymal tumours composed of histologically and immunohistochemically distinctive perivascular epitheliod cells”. It appears that PEComas may arise at any anatomical location. Angiomyolipoma is the most common pathological type, with the uterus and the kidney the most common sites. Angiomyolipoma of the liver belongs to this family. Here, we describe the case of a malignant PEComa of the liver. A 50 year-old woman was referred to our Hospital after a two weeks history of abdominal tension and pain. Physical and laboratory examinations were unremarkable. A focal lesion in the IV hepatic segment was detected by contrast enhanced ultrasonography and magnetic resonance imaging. It was diagnosed by liver biopsy and characterized by immuno-histological analysis. The PEComa was successively treated with surgical R0 excision. One year after the surgical treatment the PEComa relapsed. After a brief review of the literature, we suggest that, whether a surgical resection is performed or not, a long term follow-up should be required owing to the unpredictable behaviour of these hepatic lesions.