Syndrome in question: Gorlin-Goltz syndrome
Autor(a) principal: | |
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Data de Publicação: | 2016 |
Outros Autores: | , , , |
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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Anais brasileiros de dermatologia (Online) |
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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 |
_version_ |
1752126421357035520 |