Gorlin-goltz syndrome – case report
Autor(a) principal: | |
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Data de Publicação: | 2021 |
Outros Autores: | , , , , |
Tipo de documento: | Artigo |
Idioma: | por |
Título da fonte: | Research, Society and Development |
Texto Completo: | https://rsdjournal.org/index.php/rsd/article/view/10476 |
Resumo: | Introduction: Gorlin-Goltz syndrome is also referred to as basal cell nevus syndrome or basal cell nevoid carcinoma syndrome. This is a rare autosomal dominant inherited disorder caused by mutation in the PTCH1 tumor suppressor gene. Case report: A 15-year-old male patient sought the Maxillofacial Surgery service, reporting mild pain in the right retromolar region, and an orthopantomography was requested, noting the presence 1 maxillary injury and 2 mandible injuries (left and right), all associated with impacted teeth. In the investigation, we found calcification of the cerebral scythe, pontification of the turgical saddle, and depressions in the sole of the feet. Enucleation and curettage of maxillary lesions and removal of impacted teeth were performed. At histopathological examination, Odontogenic Keratocyst was diagnosed. Discussion: SGG is a neurocutaneous disorder that manifests mainly in young patients without gender preference. Kimonis et Al (1997) determine a form of diagnosis by the presence of larger and smaller criteria. In the reported case, three major and one minor criteria were found. It is concluded, therefore, that despite the several diagnostic findings, the most relevant for the maxillofacial surgeon are the keratocysts, which have a high recurrence and destruction power, being present in 70% of the SGG cases. more frequent in the jaw and associated with teeth included. So far, the diagnosis and treatment of the case mentioned has been satisfactory, and a multidisciplinary follow-up is also required. |
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Gorlin-goltz syndrome – case reportSíndrome de gorlin goltz – reporte de casoSíndrome de gorlin-goltz – relato de casoSíndrome de Gorlin-GoltzCeratocisto odontogênicoCistos odontogênicos.Queratoquiste odontogénicoSíndrome de Gorlin-GoltzQuistes odontogénicos.Gorlin-Goltz SyndromeOdontogenic keratocystBasal cell carcinoma.Introduction: Gorlin-Goltz syndrome is also referred to as basal cell nevus syndrome or basal cell nevoid carcinoma syndrome. This is a rare autosomal dominant inherited disorder caused by mutation in the PTCH1 tumor suppressor gene. Case report: A 15-year-old male patient sought the Maxillofacial Surgery service, reporting mild pain in the right retromolar region, and an orthopantomography was requested, noting the presence 1 maxillary injury and 2 mandible injuries (left and right), all associated with impacted teeth. In the investigation, we found calcification of the cerebral scythe, pontification of the turgical saddle, and depressions in the sole of the feet. Enucleation and curettage of maxillary lesions and removal of impacted teeth were performed. At histopathological examination, Odontogenic Keratocyst was diagnosed. Discussion: SGG is a neurocutaneous disorder that manifests mainly in young patients without gender preference. Kimonis et Al (1997) determine a form of diagnosis by the presence of larger and smaller criteria. In the reported case, three major and one minor criteria were found. It is concluded, therefore, that despite the several diagnostic findings, the most relevant for the maxillofacial surgeon are the keratocysts, which have a high recurrence and destruction power, being present in 70% of the SGG cases. more frequent in the jaw and associated with teeth included. So far, the diagnosis and treatment of the case mentioned has been satisfactory, and a multidisciplinary follow-up is also required.Introducción: El síndrome de Gorlin-Goltz también se denomina síndrome de nevo de células basales o síndrome de carcinoma nevoide de células basales. Se trata de un trastorno hereditario autosómico dominante poco común, causado por la mutación en el gen supresor de tumores PTCH1. Caso clínico: Paciente masculino de 15 años que acude al servicio de Cirugía y Traumatología Buco-Maxilofacial, refiriendo dolor leve en región retromolar derecha, solicitando ortopantomografía, constatando la presencia lesión en el maxilar y lesiones en la mandíbula, todas asociadas con dientes retenidos. En la investigación se encontró calcificación de la hoz cerebral, pontificación de la montura turca y depresiones en la región plantar de los pies. Se realizó la enucleación y legrado de las lesiones maxilares y la extracción de los dientes retenidos. El examen histopatológico reveló un queratoquiste odontogénico. Discusión: SGG consiste en un trastorno neurocutáneo, que se manifiesta principalmente en pacientes jóvenes, sin predilección de género. Kimonis et al (1997) determinan una forma de diagnóstico mediante la presencia de criterios mayores y menores. En el caso reportado, se encontraron tres criterios mayores y un criterio menor. Se concluye, por tanto, que a pesar de los diversos hallazgos diagnósticos, los más relevantes para el cirujano buccomaxilofacial son los queratoquistes, que tienen un alto poder de recurrencia y destrucción, estando presentes en el 70% de los casos de SGG, siendo más frecuente en la mandíbula y asociado a dientes incluidos. Hasta el momento, el diagnóstico y tratamiento del caso mencionado ha sido satisfactorio, requiriendo un seguimiento multiprofesional.Introdução: A síndrome de Gorlin-Goltz é também denominada de síndrome do nevo basocelular ou síndrome do carcinoma nevóide de células basais. Esta é uma rara desordem hereditária autossômica dominante, causada pela mutação no gene supressor de tumor PTCH1. Relato de caso: Paciente do gênero masculino, 15 anos de idade, procurou o serviço de Cirurgia e Traumatologia Buco-Maxilo-Facial, relatando dores discretas na região retromolar direita, sendo solicitada uma ortopantomografia, notando-se a presença de 1 lesão na maxila e 2 lesões na mandíbula (esquerda e direita), todas associadas a dentes impactados. Na investigação, encontrou-se calcificação da foice cerebral, pontificação da sela túrcica, e depressões na região plantar dos pés. Foi realizada a enucleação e curetagem das lesões maxilares e remoção dos dentes impactados. Ao exame histopatológico, foi diagnosticado Ceratocisto Odontogênico. Discussão: A SGG consiste em um transtorno neurocutâneo, que se manifesta principalmente em pacientes jovens, sem predileção por gênero. Kimonis et Al (1997) determinam uma forma de diagnóstico através da presença de critérios maiores e critérios menores. No caso relatado foi encontrado três critérios maiores e um critério menor. Conclui-se, portanto, que apesar dos diversos achados para diagnóstico, o de maior relevância para o cirurgião Buco-Maxilo-Facial são os ceratocistos, tendo um alto poder de recidiva e destruição, estando presente em 70% dos casos de SGG, sendo mais frequente na mandíbula e associado a dentes inclusos. Até o momento, o diagnóstico e tratamento do caso mencionado foi satisfatório, fazendo-se necessário também um acompanhamento multiprofissional.Research, Society and Development2021-02-25info:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersionapplication/pdfhttps://rsdjournal.org/index.php/rsd/article/view/1047610.33448/rsd-v10i2.10476Research, Society and Development; Vol. 10 No. 2; e49610210476Research, Society and Development; Vol. 10 Núm. 2; e49610210476Research, Society and Development; v. 10 n. 2; e496102104762525-3409reponame:Research, Society and Developmentinstname:Universidade Federal de Itajubá (UNIFEI)instacron:UNIFEIporhttps://rsdjournal.org/index.php/rsd/article/view/10476/11528Copyright (c) 2021 Virgílio Bernardino Ferraz Jardim; Allan Vinícius Martins de Barros; Rafaella Amorim Bittencourt Maranhão de Araújo; Lucas Viana Silva Ramos; Luiz Fernando Alves de Lima; Emanuel Dias de Oliveira e Silvahttps://creativecommons.org/licenses/by/4.0info:eu-repo/semantics/openAccessJardim, Virgílio Bernardino Ferraz Barros, Allan Vinícius Martins de Araújo, Rafaella Amorim Bittencourt Maranhão de Ramos, Lucas Viana Silva Lima, Luiz Fernando Alves de Silva, Emanuel Dias de Oliveira e 2021-03-02T09:32:39Zoai:ojs.pkp.sfu.ca:article/10476Revistahttps://rsdjournal.org/index.php/rsd/indexPUBhttps://rsdjournal.org/index.php/rsd/oairsd.articles@gmail.com2525-34092525-3409opendoar:2024-01-17T09:32:32.465828Research, Society and Development - Universidade Federal de Itajubá (UNIFEI)false |
dc.title.none.fl_str_mv |
Gorlin-goltz syndrome – case report Síndrome de gorlin goltz – reporte de caso Síndrome de gorlin-goltz – relato de caso |
title |
Gorlin-goltz syndrome – case report |
spellingShingle |
Gorlin-goltz syndrome – case report Jardim, Virgílio Bernardino Ferraz Síndrome de Gorlin-Goltz Ceratocisto odontogênico Cistos odontogênicos. Queratoquiste odontogénico Síndrome de Gorlin-Goltz Quistes odontogénicos. Gorlin-Goltz Syndrome Odontogenic keratocyst Basal cell carcinoma. |
title_short |
Gorlin-goltz syndrome – case report |
title_full |
Gorlin-goltz syndrome – case report |
title_fullStr |
Gorlin-goltz syndrome – case report |
title_full_unstemmed |
Gorlin-goltz syndrome – case report |
title_sort |
Gorlin-goltz syndrome – case report |
author |
Jardim, Virgílio Bernardino Ferraz |
author_facet |
Jardim, Virgílio Bernardino Ferraz Barros, Allan Vinícius Martins de Araújo, Rafaella Amorim Bittencourt Maranhão de Ramos, Lucas Viana Silva Lima, Luiz Fernando Alves de Silva, Emanuel Dias de Oliveira e |
author_role |
author |
author2 |
Barros, Allan Vinícius Martins de Araújo, Rafaella Amorim Bittencourt Maranhão de Ramos, Lucas Viana Silva Lima, Luiz Fernando Alves de Silva, Emanuel Dias de Oliveira e |
author2_role |
author author author author author |
dc.contributor.author.fl_str_mv |
Jardim, Virgílio Bernardino Ferraz Barros, Allan Vinícius Martins de Araújo, Rafaella Amorim Bittencourt Maranhão de Ramos, Lucas Viana Silva Lima, Luiz Fernando Alves de Silva, Emanuel Dias de Oliveira e |
dc.subject.por.fl_str_mv |
Síndrome de Gorlin-Goltz Ceratocisto odontogênico Cistos odontogênicos. Queratoquiste odontogénico Síndrome de Gorlin-Goltz Quistes odontogénicos. Gorlin-Goltz Syndrome Odontogenic keratocyst Basal cell carcinoma. |
topic |
Síndrome de Gorlin-Goltz Ceratocisto odontogênico Cistos odontogênicos. Queratoquiste odontogénico Síndrome de Gorlin-Goltz Quistes odontogénicos. Gorlin-Goltz Syndrome Odontogenic keratocyst Basal cell carcinoma. |
description |
Introduction: Gorlin-Goltz syndrome is also referred to as basal cell nevus syndrome or basal cell nevoid carcinoma syndrome. This is a rare autosomal dominant inherited disorder caused by mutation in the PTCH1 tumor suppressor gene. Case report: A 15-year-old male patient sought the Maxillofacial Surgery service, reporting mild pain in the right retromolar region, and an orthopantomography was requested, noting the presence 1 maxillary injury and 2 mandible injuries (left and right), all associated with impacted teeth. In the investigation, we found calcification of the cerebral scythe, pontification of the turgical saddle, and depressions in the sole of the feet. Enucleation and curettage of maxillary lesions and removal of impacted teeth were performed. At histopathological examination, Odontogenic Keratocyst was diagnosed. Discussion: SGG is a neurocutaneous disorder that manifests mainly in young patients without gender preference. Kimonis et Al (1997) determine a form of diagnosis by the presence of larger and smaller criteria. In the reported case, three major and one minor criteria were found. It is concluded, therefore, that despite the several diagnostic findings, the most relevant for the maxillofacial surgeon are the keratocysts, which have a high recurrence and destruction power, being present in 70% of the SGG cases. more frequent in the jaw and associated with teeth included. So far, the diagnosis and treatment of the case mentioned has been satisfactory, and a multidisciplinary follow-up is also required. |
publishDate |
2021 |
dc.date.none.fl_str_mv |
2021-02-25 |
dc.type.driver.fl_str_mv |
info:eu-repo/semantics/article info:eu-repo/semantics/publishedVersion |
format |
article |
status_str |
publishedVersion |
dc.identifier.uri.fl_str_mv |
https://rsdjournal.org/index.php/rsd/article/view/10476 10.33448/rsd-v10i2.10476 |
url |
https://rsdjournal.org/index.php/rsd/article/view/10476 |
identifier_str_mv |
10.33448/rsd-v10i2.10476 |
dc.language.iso.fl_str_mv |
por |
language |
por |
dc.relation.none.fl_str_mv |
https://rsdjournal.org/index.php/rsd/article/view/10476/11528 |
dc.rights.driver.fl_str_mv |
https://creativecommons.org/licenses/by/4.0 info:eu-repo/semantics/openAccess |
rights_invalid_str_mv |
https://creativecommons.org/licenses/by/4.0 |
eu_rights_str_mv |
openAccess |
dc.format.none.fl_str_mv |
application/pdf |
dc.publisher.none.fl_str_mv |
Research, Society and Development |
publisher.none.fl_str_mv |
Research, Society and Development |
dc.source.none.fl_str_mv |
Research, Society and Development; Vol. 10 No. 2; e49610210476 Research, Society and Development; Vol. 10 Núm. 2; e49610210476 Research, Society and Development; v. 10 n. 2; e49610210476 2525-3409 reponame:Research, Society and Development instname:Universidade Federal de Itajubá (UNIFEI) instacron:UNIFEI |
instname_str |
Universidade Federal de Itajubá (UNIFEI) |
instacron_str |
UNIFEI |
institution |
UNIFEI |
reponame_str |
Research, Society and Development |
collection |
Research, Society and Development |
repository.name.fl_str_mv |
Research, Society and Development - Universidade Federal de Itajubá (UNIFEI) |
repository.mail.fl_str_mv |
rsd.articles@gmail.com |
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1797052665070354432 |