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...

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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
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author Idania Lubo
Ileana Fermín
Olindo Massarelli
Roberta Gobbi
Paolo Cossu Rocca
author_facet Idania Lubo
Ileana Fermín
Olindo Massarelli
Roberta Gobbi
Paolo Cossu Rocca
author_sort Idania Lubo
collection DOAJ
description 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.
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spelling doaj-art-402ec06170c54bcaa8fdffa6928f8a962025-08-20T02:39:15ZengWileyCase Reports in Pathology2090-67812090-679X2016-01-01201610.1155/2016/19364211936421Perivascular Epithelioid Cell Tumour with Intraorbital Location: Report of a Case and Review of the LiteratureIdania Lubo0Ileana Fermín1Olindo Massarelli2Roberta Gobbi3Paolo Cossu Rocca4Anatomy and Pathology Institute “Dr. José Antonio O’Daly”, Central University of Venezuela, Los Chaguaramos 1040, Caracas, VenezuelaAnatomy and Pathology Institute “Dr. José Antonio O’Daly”, Central University of Venezuela, Los Chaguaramos 1040, Caracas, VenezuelaMaxillofacial Surgery Unit, University of Sassari, ItalyMaxillofacial Surgery Unit, University of Sassari, ItalyDepartment of Histopathology and Anatomical Pathology, University of Sassari, ItalyThe 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.http://dx.doi.org/10.1155/2016/1936421
spellingShingle Idania Lubo
Ileana Fermín
Olindo Massarelli
Roberta Gobbi
Paolo Cossu Rocca
Perivascular Epithelioid Cell Tumour with Intraorbital Location: Report of a Case and Review of the Literature
Case Reports in Pathology
title Perivascular Epithelioid Cell Tumour with Intraorbital Location: Report of a Case and Review of the Literature
title_full Perivascular Epithelioid Cell Tumour with Intraorbital Location: Report of a Case and Review of the Literature
title_fullStr Perivascular Epithelioid Cell Tumour with Intraorbital Location: Report of a Case and Review of the Literature
title_full_unstemmed Perivascular Epithelioid Cell Tumour with Intraorbital Location: Report of a Case and Review of the Literature
title_short Perivascular Epithelioid Cell Tumour with Intraorbital Location: Report of a Case and Review of the Literature
title_sort perivascular epithelioid cell tumour with intraorbital location report of a case and review of the literature
url http://dx.doi.org/10.1155/2016/1936421
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