Syndrome in question: Gorlin-Goltz syndrome

Detalhes bibliográficos
Autor(a) principal: Ribeiro,Pauline Lyrio
Data de Publicação: 2016
Outros Autores: Souza Filho,João Basílio de, Abreu,Karina Demoner de, Brezinscki,Marisa Simon, Pignaton,Christine Chambo
Tipo de documento: Relatório
Idioma: eng
Título da fonte: Anais brasileiros de dermatologia (Online)
Texto Completo: http://old.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962016000400541
Resumo: Abstract: The Nevoid Basal Cell Carcinoma Syndrome (NBCCS) is an uncommon disorder caused by a mutation in Patched, tumor suppressor gene. It is mainly characterized by numerous early onset basal cell carcinomas, odontogenic cysts of jaw and skeletal abnormalities. Due to the wide clinical spectrum, treatment and management of its modalities are not standardized and should be individualized and monitored by a multidisciplinary team. We report a typical case in a 30-year-old man with multiple basal cell carcinomas, keratotic pits of palmar creases and bifid ribs, with a history of several corrective surgeries for keratocystic odontogenic tumors, among other lesions characteristic of the syndrome.
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spelling Syndrome in question: Gorlin-Goltz syndromeBasal cell nevus syndromeCarcinoma, basal cellOdontogenic cystsAbstract: The Nevoid Basal Cell Carcinoma Syndrome (NBCCS) is an uncommon disorder caused by a mutation in Patched, tumor suppressor gene. It is mainly characterized by numerous early onset basal cell carcinomas, odontogenic cysts of jaw and skeletal abnormalities. Due to the wide clinical spectrum, treatment and management of its modalities are not standardized and should be individualized and monitored by a multidisciplinary team. We report a typical case in a 30-year-old man with multiple basal cell carcinomas, keratotic pits of palmar creases and bifid ribs, with a history of several corrective surgeries for keratocystic odontogenic tumors, among other lesions characteristic of the syndrome.Sociedade Brasileira de Dermatologia2016-08-01info:eu-repo/semantics/reportinfo:eu-repo/semantics/publishedVersiontext/htmlhttp://old.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962016000400541Anais Brasileiros de Dermatologia v.91 n.4 2016reponame:Anais brasileiros de dermatologia (Online)instname:Sociedade Brasileira de Dermatologia (SBD)instacron:SBD10.1590/abd1806-4841.20164428info:eu-repo/semantics/openAccessRibeiro,Pauline LyrioSouza Filho,João Basílio deAbreu,Karina Demoner deBrezinscki,Marisa SimonPignaton,Christine Chamboeng2016-08-26T00:00:00Zoai:scielo:S0365-05962016000400541Revistahttp://www.anaisdedermatologia.org.br/https://old.scielo.br/oai/scielo-oai.phpabd@sbd.org.br||revista@sbd.org.br1806-48410365-0596opendoar:2016-08-26T00:00Anais brasileiros de dermatologia (Online) - Sociedade Brasileira de Dermatologia (SBD)false
dc.title.none.fl_str_mv Syndrome in question: Gorlin-Goltz syndrome
title Syndrome in question: Gorlin-Goltz syndrome
spellingShingle Syndrome in question: Gorlin-Goltz syndrome
Ribeiro,Pauline Lyrio
Basal cell nevus syndrome
Carcinoma, basal cell
Odontogenic cysts
title_short Syndrome in question: Gorlin-Goltz syndrome
title_full Syndrome in question: Gorlin-Goltz syndrome
title_fullStr Syndrome in question: Gorlin-Goltz syndrome
title_full_unstemmed Syndrome in question: Gorlin-Goltz syndrome
title_sort Syndrome in question: Gorlin-Goltz syndrome
author Ribeiro,Pauline Lyrio
author_facet Ribeiro,Pauline Lyrio
Souza Filho,João Basílio de
Abreu,Karina Demoner de
Brezinscki,Marisa Simon
Pignaton,Christine Chambo
author_role author
author2 Souza Filho,João Basílio de
Abreu,Karina Demoner de
Brezinscki,Marisa Simon
Pignaton,Christine Chambo
author2_role author
author
author
author
dc.contributor.author.fl_str_mv Ribeiro,Pauline Lyrio
Souza Filho,João Basílio de
Abreu,Karina Demoner de
Brezinscki,Marisa Simon
Pignaton,Christine Chambo
dc.subject.por.fl_str_mv Basal cell nevus syndrome
Carcinoma, basal cell
Odontogenic cysts
topic Basal cell nevus syndrome
Carcinoma, basal cell
Odontogenic cysts
description Abstract: The Nevoid Basal Cell Carcinoma Syndrome (NBCCS) is an uncommon disorder caused by a mutation in Patched, tumor suppressor gene. It is mainly characterized by numerous early onset basal cell carcinomas, odontogenic cysts of jaw and skeletal abnormalities. Due to the wide clinical spectrum, treatment and management of its modalities are not standardized and should be individualized and monitored by a multidisciplinary team. We report a typical case in a 30-year-old man with multiple basal cell carcinomas, keratotic pits of palmar creases and bifid ribs, with a history of several corrective surgeries for keratocystic odontogenic tumors, among other lesions characteristic of the syndrome.
publishDate 2016
dc.date.none.fl_str_mv 2016-08-01
dc.type.driver.fl_str_mv info:eu-repo/semantics/report
dc.type.status.fl_str_mv info:eu-repo/semantics/publishedVersion
format report
status_str publishedVersion
dc.identifier.uri.fl_str_mv http://old.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962016000400541
url http://old.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962016000400541
dc.language.iso.fl_str_mv eng
language eng
dc.relation.none.fl_str_mv 10.1590/abd1806-4841.20164428
dc.rights.driver.fl_str_mv info:eu-repo/semantics/openAccess
eu_rights_str_mv openAccess
dc.format.none.fl_str_mv text/html
dc.publisher.none.fl_str_mv Sociedade Brasileira de Dermatologia
publisher.none.fl_str_mv Sociedade Brasileira de Dermatologia
dc.source.none.fl_str_mv Anais Brasileiros de Dermatologia v.91 n.4 2016
reponame:Anais brasileiros de dermatologia (Online)
instname:Sociedade Brasileira de Dermatologia (SBD)
instacron:SBD
instname_str Sociedade Brasileira de Dermatologia (SBD)
instacron_str SBD
institution SBD
reponame_str Anais brasileiros de dermatologia (Online)
collection Anais brasileiros de dermatologia (Online)
repository.name.fl_str_mv Anais brasileiros de dermatologia (Online) - Sociedade Brasileira de Dermatologia (SBD)
repository.mail.fl_str_mv abd@sbd.org.br||revista@sbd.org.br
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