Gorlin-Goltz syndrome : Clinicopathologic aspects
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Gorlin-Goltz syndrome : Clinicopathologic aspects

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Gorlin-Goltz syndrome : Clinicopathologic aspects

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dc.contributor.author Ortega García de Amezaga, Aitziber es
dc.contributor.author García Arregui, Olatz es
dc.contributor.author Zepeda Nuño, Sergio es
dc.contributor.author Acha Sagredo, Amelia es
dc.contributor.author Aguirre Urízar, José Manuel es
dc.date.accessioned 2017-09-15T07:55:08Z
dc.date.available 2017-09-15T07:55:08Z
dc.date.issued 2008 es
dc.identifier.uri http://hdl.handle.net/10550/60839
dc.description.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 clinicopathologic and the therapeutic aspects related to the syndrome under consideration have been revised and updated. es
dc.source Ortega García de Amezaga, Aitziber ; García Arregui, Olatz ; Zepeda Nuño, Sergio ; Acha Sagredo, Amelia ; Aguirre Urízar, José Manuel. Gorlin-Goltz syndrome : Clinicopathologic aspects. En: Medicina oral, patología oral y cirugía bucal. Ed. inglesa, 13 6 2008: 2- es
dc.title Gorlin-Goltz syndrome : Clinicopathologic aspects es
dc.type info:eu-repo/semantics/article en
dc.type info:eu-repo/semantics/publishedVersion en
dc.subject.unesco UNESCO::CIENCIAS MÉDICAS es
dc.identifier.doi es

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