Gorlin syndrome: Importance of clinical signs and danger of delayed diagnosis A case report with eight years follow-up
Autor(a) principal: | |
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Data de Publicação: | 2014 |
Outros Autores: | , , , |
Tipo de documento: | Artigo |
Idioma: | eng |
Título da fonte: | Repositório Institucional da UNESP |
Texto Completo: | http://www.scopemed.org/?mno=43373 http://hdl.handle.net/11449/125609 |
Resumo: | Nevoid basal cell carcinoma (NBCCS) or Gorlin-Goltz syndrome (GS) is a multidisciplinary problem, the early diagnosis of which allows secondary prophylaxis that follows an appropriate regimen to delay progression of the syndrome. The aim of this study was to present a case of delayed diagnosis of GS in a young patient who received multidisciplinary treatment 5 years after onset. The patient presented for evaluation with painless swelling of the left maxilla. Histological examination confirmed the diagnosis of a keratocyst odontogenic tumor (KOT) that was enucleated. On presentation, the patient’s symptoms and clinical signs were not related to complications of GS, and the possibility of GS was initially rejected, as he did not have a family history of the syndrome. Four years after the first surgery to remove the lesion, the patient came to our clinic with a brown, pigmented lesion. Computed tomography revealed ectopic lamellar calcification of the falx cerebri, which was the conclusive factor for the diagnosis of GS. It is important that clinicians recognize the clinical signs of GS, which mainly manifests itself as multiple basal cell carcinomas in the skin. |
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Gorlin syndrome: Importance of clinical signs and danger of delayed diagnosis A case report with eight years follow-upBasal cell nevus syndromeGorlin syndromeKeratocyst odontogenic tumorSindrome de GorlinDiferencial diagnosesNevoid basal cell carcinoma (NBCCS) or Gorlin-Goltz syndrome (GS) is a multidisciplinary problem, the early diagnosis of which allows secondary prophylaxis that follows an appropriate regimen to delay progression of the syndrome. The aim of this study was to present a case of delayed diagnosis of GS in a young patient who received multidisciplinary treatment 5 years after onset. The patient presented for evaluation with painless swelling of the left maxilla. Histological examination confirmed the diagnosis of a keratocyst odontogenic tumor (KOT) that was enucleated. On presentation, the patient’s symptoms and clinical signs were not related to complications of GS, and the possibility of GS was initially rejected, as he did not have a family history of the syndrome. Four years after the first surgery to remove the lesion, the patient came to our clinic with a brown, pigmented lesion. Computed tomography revealed ectopic lamellar calcification of the falx cerebri, which was the conclusive factor for the diagnosis of GS. It is important that clinicians recognize the clinical signs of GS, which mainly manifests itself as multiple basal cell carcinomas in the skin.Universidade Estadual Paulista Júlio de Mesquita Filho, Departamento de Diagnóstico e Cirurgia, Faculdade de Odontologia de Araraquara, Araraquara, Rua Humaitá, 1680, Centro, CEP 14801-903, SP, BrasilUniversidade Estadual Paulista Júlio de Mesquita Filho, Departamento de Diagnóstico e Cirurgia, Faculdade de Odontologia de Araraquara, Araraquara, Rua Humaitá, 1680, Centro, CEP 14801-903, SP, BrasilUniversidade Estadual Paulista (Unesp)Avila, Erica [UNESP]Molon, Rafael [UNESP]Gabrielli, Mario [UNESP]Vieira, Eduardo Hochuli [UNESP]Gabrielli, Marisa [UNESP]2015-08-06T16:12:36Z2015-08-06T16:12:36Z2014info:eu-repo/semantics/publishedVersioninfo:eu-repo/semantics/article1-4application/pdfhttp://www.scopemed.org/?mno=43373Archives of Clinical and Experimental Surgery, v. 1, p. 1-4, 2014.2146-8133http://hdl.handle.net/11449/12560910.5455/aces.20140108034044ISSN2146-8133-2014-01-01-04.pdf685348548368367880291771699165258492596401380580Currículo Lattesreponame:Repositório Institucional da UNESPinstname:Universidade Estadual Paulista (UNESP)instacron:UNESPengArchives of Clinical and Experimental Surgeryinfo:eu-repo/semantics/openAccess2024-01-12T06:28:40Zoai:repositorio.unesp.br:11449/125609Repositório InstitucionalPUBhttp://repositorio.unesp.br/oai/requestopendoar:29462024-01-12T06:28:40Repositório Institucional da UNESP - Universidade Estadual Paulista (UNESP)false |
dc.title.none.fl_str_mv |
Gorlin syndrome: Importance of clinical signs and danger of delayed diagnosis A case report with eight years follow-up |
title |
Gorlin syndrome: Importance of clinical signs and danger of delayed diagnosis A case report with eight years follow-up |
spellingShingle |
Gorlin syndrome: Importance of clinical signs and danger of delayed diagnosis A case report with eight years follow-up Avila, Erica [UNESP] Basal cell nevus syndrome Gorlin syndrome Keratocyst odontogenic tumor Sindrome de Gorlin Diferencial diagnoses |
title_short |
Gorlin syndrome: Importance of clinical signs and danger of delayed diagnosis A case report with eight years follow-up |
title_full |
Gorlin syndrome: Importance of clinical signs and danger of delayed diagnosis A case report with eight years follow-up |
title_fullStr |
Gorlin syndrome: Importance of clinical signs and danger of delayed diagnosis A case report with eight years follow-up |
title_full_unstemmed |
Gorlin syndrome: Importance of clinical signs and danger of delayed diagnosis A case report with eight years follow-up |
title_sort |
Gorlin syndrome: Importance of clinical signs and danger of delayed diagnosis A case report with eight years follow-up |
author |
Avila, Erica [UNESP] |
author_facet |
Avila, Erica [UNESP] Molon, Rafael [UNESP] Gabrielli, Mario [UNESP] Vieira, Eduardo Hochuli [UNESP] Gabrielli, Marisa [UNESP] |
author_role |
author |
author2 |
Molon, Rafael [UNESP] Gabrielli, Mario [UNESP] Vieira, Eduardo Hochuli [UNESP] Gabrielli, Marisa [UNESP] |
author2_role |
author author author author |
dc.contributor.none.fl_str_mv |
Universidade Estadual Paulista (Unesp) |
dc.contributor.author.fl_str_mv |
Avila, Erica [UNESP] Molon, Rafael [UNESP] Gabrielli, Mario [UNESP] Vieira, Eduardo Hochuli [UNESP] Gabrielli, Marisa [UNESP] |
dc.subject.por.fl_str_mv |
Basal cell nevus syndrome Gorlin syndrome Keratocyst odontogenic tumor Sindrome de Gorlin Diferencial diagnoses |
topic |
Basal cell nevus syndrome Gorlin syndrome Keratocyst odontogenic tumor Sindrome de Gorlin Diferencial diagnoses |
description |
Nevoid basal cell carcinoma (NBCCS) or Gorlin-Goltz syndrome (GS) is a multidisciplinary problem, the early diagnosis of which allows secondary prophylaxis that follows an appropriate regimen to delay progression of the syndrome. The aim of this study was to present a case of delayed diagnosis of GS in a young patient who received multidisciplinary treatment 5 years after onset. The patient presented for evaluation with painless swelling of the left maxilla. Histological examination confirmed the diagnosis of a keratocyst odontogenic tumor (KOT) that was enucleated. On presentation, the patient’s symptoms and clinical signs were not related to complications of GS, and the possibility of GS was initially rejected, as he did not have a family history of the syndrome. Four years after the first surgery to remove the lesion, the patient came to our clinic with a brown, pigmented lesion. Computed tomography revealed ectopic lamellar calcification of the falx cerebri, which was the conclusive factor for the diagnosis of GS. It is important that clinicians recognize the clinical signs of GS, which mainly manifests itself as multiple basal cell carcinomas in the skin. |
publishDate |
2014 |
dc.date.none.fl_str_mv |
2014 2015-08-06T16:12:36Z 2015-08-06T16:12:36Z |
dc.type.status.fl_str_mv |
info:eu-repo/semantics/publishedVersion |
dc.type.driver.fl_str_mv |
info:eu-repo/semantics/article |
format |
article |
status_str |
publishedVersion |
dc.identifier.uri.fl_str_mv |
http://www.scopemed.org/?mno=43373 Archives of Clinical and Experimental Surgery, v. 1, p. 1-4, 2014. 2146-8133 http://hdl.handle.net/11449/125609 10.5455/aces.20140108034044 ISSN2146-8133-2014-01-01-04.pdf 6853485483683678 8029177169916525 8492596401380580 |
url |
http://www.scopemed.org/?mno=43373 http://hdl.handle.net/11449/125609 |
identifier_str_mv |
Archives of Clinical and Experimental Surgery, v. 1, p. 1-4, 2014. 2146-8133 10.5455/aces.20140108034044 ISSN2146-8133-2014-01-01-04.pdf 6853485483683678 8029177169916525 8492596401380580 |
dc.language.iso.fl_str_mv |
eng |
language |
eng |
dc.relation.none.fl_str_mv |
Archives of Clinical and Experimental Surgery |
dc.rights.driver.fl_str_mv |
info:eu-repo/semantics/openAccess |
eu_rights_str_mv |
openAccess |
dc.format.none.fl_str_mv |
1-4 application/pdf |
dc.source.none.fl_str_mv |
Currículo Lattes reponame:Repositório Institucional da UNESP instname:Universidade Estadual Paulista (UNESP) instacron:UNESP |
instname_str |
Universidade Estadual Paulista (UNESP) |
instacron_str |
UNESP |
institution |
UNESP |
reponame_str |
Repositório Institucional da UNESP |
collection |
Repositório Institucional da UNESP |
repository.name.fl_str_mv |
Repositório Institucional da UNESP - Universidade Estadual Paulista (UNESP) |
repository.mail.fl_str_mv |
|
_version_ |
1799965598293164032 |