Gorlin-goltz syndrome – case report

Detalhes bibliográficos
Autor(a) principal: Jardim, Virgílio Bernardino Ferraz
Data de Publicação: 2021
Outros Autores: 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
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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spelling 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
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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
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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)
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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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