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

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...

Full description

Saved in:
Bibliographic Details
Main Authors: Idania Lubo, Ileana Fermín, Olindo Massarelli, Roberta Gobbi, Paolo Cossu Rocca
Format: Article
Language:English
Published: Wiley 2016-01-01
Series:Case Reports in Pathology
Online Access:http://dx.doi.org/10.1155/2016/1936421
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary: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.
ISSN:2090-6781
2090-679X