Primary perivascular epithelioid cell tumor of the liver not related to hepatic ligaments: Hepatic PEComa as an emerging entity
Arthur Zimmermann1, Christian von der Brelie2, Barbara Berger1, Andreas Kappeler1 and Daniel Candinas3
1Institute of Pathology of the University Berne, Switzerland, 2Clinic and Policlinic of Neurosurgery, Universitätsklinikum, Bonn, Germany, and 3Department of Visceral and Transplantation Surgery, University Hospital, Berne, Switzerland.
Offprint requests to: Arthur Zimmermann, M.D. Institute of Pathology of the University, Murtenstrasse 31, CH-3010 Berne, Switzerland. e-mail: zimmerma@patho.unibe.ch
Summary. Primary perivascular epithelioid cell tumor (PEComa) of the liver is a very rare example of an emerging family of hepatic PEC tumors. Only few cases have been described so far. We report the case of a large but benign hepatic PEComa in a 53-year-old man without signs of tuberous sclerosis. In contrast to recently described PEC-derived liver tumors in children and young adults, this neoplasm was not related to the hepatic ligaments but had developed deeply within the liver substance. The neoplastic cells displayed the complete phenotype typical for PEComas, i.e. reactivity for several melanoma markers and for smooth muscle actin. The unique relationship of myoid tumor cells to the adventitia of blood vessels prompted us, in comparison with published findings obtained with angiomyolipomas, to comment on the possible origin of the still enigmatic perivascular epithelioid cells. Histol Histopathol 23, 1185-1193 (2008)
Key words: Perivascular epithelioid cell tumor, PEComa, Liver
DOI: 10.14670/HH-23.1185