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Case Reports in Pathology
Volume 2016 (2016), Article ID 1936421, 6 pages
http://dx.doi.org/10.1155/2016/1936421
Case Report

Perivascular Epithelioid Cell Tumour with Intraorbital Location: Report of a Case and Review of the Literature

1Anatomy and Pathology Institute “Dr. José Antonio O’Daly”, Central University of Venezuela, Los Chaguaramos 1040, Caracas, Venezuela
2Maxillofacial Surgery Unit, University of Sassari, Italy
3Department of Histopathology and Anatomical Pathology, University of Sassari, Italy

Received 30 August 2015; Accepted 12 November 2015

Academic Editor: Maria M. Picken

Copyright © 2016 Idania Lubo et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

The Perivascular Epithelioid Cell tumours (PEComas) are rare mesenchymal neoplasms recognized as entity by the World Health Organization. The tumour cells have an uncertain origin and are characterized by distinctive histological and immunohistochemical features. We report a case of PEComa occurring as intraorbital lesion in a 47-year-old man. We found only two other cases described in the literature and we considered all three cases together in order of histology, immunohistochemistry, and clinical outcome. We found a strict histological overlapping and quite similar immunohistological results. All three cases showed a favourable clinical course probably related to small size of tumours (<5 cm), low mitotic rate (<2 mitoses in 50 HPF), and absence of necrosis.