PEComa of the nose: report of a case with immunohistochemical and ultrustructural studies and a review of the literature.
Head Neck Pathol
; 8(1): 122-6, 2014 Mar.
Article
en En
| MEDLINE
| ID: mdl-23838855
ABSTRACT
PEComas are a family of mesenchymal neoplasms that have in common the presence of a unique cell type, the perivascular epithelioid cell (PEC). PECs uniquely exhibit a distinct immunophenotype with expression of both melanocytic, particularly HMB-45, and myogenic markers. Nasal PEComas are exceedingly rare. To date, 14 cases have been described in the literature and with the exception of 6 cases, the rest consistently lack epithelioid cells and HMB-45 expression and are best classified as nasal hamartomas or angioleiomyomas with an adipocytic component. Nasal PEComas may closely resemble malignant melanomas since both entities share many morphologic, immunohistochemical, ultrastructural and clinical features. The distinction is of paramount importance as melanomas tend to display an aggressive behaviour with associated poor outcome. Herein, we report a case of nasal PEComa in a 19 year girl, focusing on the importance of light microscopic, immunohistochemical and ultrastructural features in accurately establishing the diagnosis.
Texto completo:
1
Colección:
01-internacional
Banco de datos:
MEDLINE
Asunto principal:
Neoplasias Nasales
/
Neoplasias de Células Epitelioides Perivasculares
Límite:
Adult
/
Female
/
Humans
Idioma:
En
Revista:
Head Neck Pathol
Asunto de la revista:
NEOPLASIAS
/
PATOLOGIA
Año:
2014
Tipo del documento:
Article