Gorlin-Goltz syndrome: Clinicopathologic aspects
Med. oral patol. oral cir. bucal (Internet)
; 13(6): 338-343, jun. 2008. ilus, tab
Artigo
em En
| IBECS
| ID: ibc-67408
Biblioteca responsável:
ES15.1
Localização: ES15.1 - BNCS
ABSTRACT
Gorlin-Goltz syndrome, also known as nevoid basal cell carcicoma syndrome, comes into being due to a genetic alteration produced by a mutation in the Patched tumour suppressor gene, and it is inherited in a dominant autosomal way, though sporadic cases have been found. This syndrome shows a high penetrance and variable expressiveness. It is about a multisystemic process that is characterised by the presence of multiple pigmented basocellular carcinomas, keratocysts in the jaws, palmar and/or plantar pits and calcification of the falxcerebri. Together with these major features a great number of processes considered as minor features have also been described. The latter include numerous skeletical, dermatology related and neurological anomalies among others. In some occasions, the presence of very aggressive basocellular carcinomas has been described as well as other malignant neoplasias. Due to the importance of oral maxillofacial manifestations of this syndrome, it is fundamental to know its characteristic in order to make a diagnosis, an early preventive treatment and establish right genetic advice. In this work the main clinicopathologicand the therapeutic aspects related to the syndrome under consideration have been revised and updated
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Espanha
Base de dados:
IBECS
Assunto principal:
Síndrome do Nevo Basocelular
Limite:
Humanos
Idioma:
Inglês
Revista:
Med. oral patol. oral cir. bucal (Internet)
Ano de publicação:
2008
Tipo de documento:
Artigo
Instituição/País de afiliação:
University of País Vasco/Spain