Multiple endocrine neoplasm type 2b - Report of three cases and review of the literature

Detalhes bibliográficos
Autor(a) principal: Urban, Cícero de Andrade
Data de Publicação: 2022
Outros Autores: Sluminsky , Beatriz Garcia, Dellê, Linei Augusta Brollini, Cury , Antônio Moris, Carvalho, José Gastão Rocha de, Hakim Neto, Calixto Antonio
Tipo de documento: Artigo
Idioma: por
Título da fonte: Revista Brasileira de Cancerologia (Online)
Texto Completo: https://rbc.inca.gov.br/index.php/revista/article/view/2776
Resumo: Multiple Endocrine Neoplasia lype ílb (MEN-IIh) is an uncommon syndrome inherited in na autossomal dominant pattern, with 100% penetrance and variable expression. This syndrome is marked by the presence of medullary thyroid carcinoma, pheocromociíoma, and diffuse ganglioneuromatosis of the gastrointestinal tract. Patients have a characteristic fades and marfanoid habitas. The autors report three cases of MEN-IIb, two of them are male, with 15,9 years on average. The first clinical expression in all the cases was the growing cervical mass on the site of the thyroid place and marfanoid habitas. The patients underwent total thyroidectomy with bilateral neck dissection for medullary thyroid carcinoma. One patient had pheochromocitoma fouryears after the diagnosis of the thyroid tumor, and underwent surgical treatment. One patient died 2,5 years after diagnosis, and the others are free of ilness one year after diagnosis.
id INCA-1_5cc7b80999747da4c1595c75f5a3d226
oai_identifier_str oai:rbc.inca.gov.br:article/2776
network_acronym_str INCA-1
network_name_str Revista Brasileira de Cancerologia (Online)
repository_id_str
spelling Multiple endocrine neoplasm type 2b - Report of three cases and review of the literatureNeoplasia Endócrína Múltipla tipo 2b - Relato de três casos e revisão de LiteraturaCarcinoma Medular de TireóideFeocromocitomaNeoplasia Endócrina MúltiplaMedullary Carcinoma of ThyroidPheochromocitomaMultiple Endocrine NeoplasiaMultiple Endocrine Neoplasia lype ílb (MEN-IIh) is an uncommon syndrome inherited in na autossomal dominant pattern, with 100% penetrance and variable expression. This syndrome is marked by the presence of medullary thyroid carcinoma, pheocromociíoma, and diffuse ganglioneuromatosis of the gastrointestinal tract. Patients have a characteristic fades and marfanoid habitas. The autors report three cases of MEN-IIb, two of them are male, with 15,9 years on average. The first clinical expression in all the cases was the growing cervical mass on the site of the thyroid place and marfanoid habitas. The patients underwent total thyroidectomy with bilateral neck dissection for medullary thyroid carcinoma. One patient had pheochromocitoma fouryears after the diagnosis of the thyroid tumor, and underwent surgical treatment. One patient died 2,5 years after diagnosis, and the others are free of ilness one year after diagnosis.Neoplasia Endocrina Múltipla tipo 2B (NEM-2B) é uma síndrome incomum, herdada de forma autossômica dominante e com penetrância variável. Múltiplos órgãos são envolvidos, mas com uma tríade predominante de carcinoma medular de tireóide, feocromocitoma e ganglioneuromas do trato gastrointestinal. A maioria dos pacientes tem fácies característica, composta por eversão e alargamento dos lábios e eversão das pálpebras por neuromas mucosos. Os autores relatam três casos de NEM-2B, sendo dois do sexo masculino, com idade média de 15,9 anos. A manifestação clínica inicial em todos os casos foi crescimento de massa cervical em topografia de tireóide e hábito marfanóide. Foram submetidos a tireoidectomia total, com esvaziamento cervical bilateral por carcinoma medular de tireóide. Um paciente apresentou feocromocitoma quatro anos após o diagnóstico de tumor de tireóide, sendo submetido a laparotomia com excisão do tumor. Um paciente evoluiu a óbito 2,5 anos após o diagnóstico e os outros dois encontram-se livres de doença, com uma média de acompanhamento ambulatorial de um ano, apesar do teste de calcitonina positivo em um dos casos.INCA2022-09-21info:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersionRelato de Casoapplication/pdfhttps://rbc.inca.gov.br/index.php/revista/article/view/277610.32635/2176-9745.RBC.1999v45n2.2776Revista Brasileira de Cancerologia; Vol. 45 No. 2 (1999): Apr./May/June; 47-53Revista Brasileira de Cancerologia; Vol. 45 Núm. 2 (1999): abr./mayo/jun.; 47-53Revista Brasileira de Cancerologia; v. 45 n. 2 (1999): abr./maio/jun.; 47-532176-9745reponame:Revista Brasileira de Cancerologia (Online)instname:Instituto Nacional de Câncer José Alencar Gomes da Silva (INCA)instacron:INCAporhttps://rbc.inca.gov.br/index.php/revista/article/view/2776/1653https://creativecommons.org/licenses/by/4.0info:eu-repo/semantics/openAccessUrban, Cícero de Andrade Sluminsky , Beatriz Garcia Dellê, Linei Augusta Brollini Cury , Antônio Moris Carvalho, José Gastão Rocha de Hakim Neto, Calixto Antonio 2023-01-18T15:14:10Zoai:rbc.inca.gov.br:article/2776Revistahttps://rbc.inca.gov.br/index.php/revistaPUBhttps://rbc.inca.gov.br/index.php/revista/oairbc@inca.gov.br0034-71162176-9745opendoar:2023-01-18T15:14:10Revista Brasileira de Cancerologia (Online) - Instituto Nacional de Câncer José Alencar Gomes da Silva (INCA)false
dc.title.none.fl_str_mv Multiple endocrine neoplasm type 2b - Report of three cases and review of the literature
Neoplasia Endócrína Múltipla tipo 2b - Relato de três casos e revisão de Literatura
title Multiple endocrine neoplasm type 2b - Report of three cases and review of the literature
spellingShingle Multiple endocrine neoplasm type 2b - Report of three cases and review of the literature
Urban, Cícero de Andrade
Carcinoma Medular de Tireóide
Feocromocitoma
Neoplasia Endócrina Múltipla
Medullary Carcinoma of Thyroid
Pheochromocitoma
Multiple Endocrine Neoplasia
title_short Multiple endocrine neoplasm type 2b - Report of three cases and review of the literature
title_full Multiple endocrine neoplasm type 2b - Report of three cases and review of the literature
title_fullStr Multiple endocrine neoplasm type 2b - Report of three cases and review of the literature
title_full_unstemmed Multiple endocrine neoplasm type 2b - Report of three cases and review of the literature
title_sort Multiple endocrine neoplasm type 2b - Report of three cases and review of the literature
author Urban, Cícero de Andrade
author_facet Urban, Cícero de Andrade
Sluminsky , Beatriz Garcia
Dellê, Linei Augusta Brollini
Cury , Antônio Moris
Carvalho, José Gastão Rocha de
Hakim Neto, Calixto Antonio
author_role author
author2 Sluminsky , Beatriz Garcia
Dellê, Linei Augusta Brollini
Cury , Antônio Moris
Carvalho, José Gastão Rocha de
Hakim Neto, Calixto Antonio
author2_role author
author
author
author
author
dc.contributor.author.fl_str_mv Urban, Cícero de Andrade
Sluminsky , Beatriz Garcia
Dellê, Linei Augusta Brollini
Cury , Antônio Moris
Carvalho, José Gastão Rocha de
Hakim Neto, Calixto Antonio
dc.subject.por.fl_str_mv Carcinoma Medular de Tireóide
Feocromocitoma
Neoplasia Endócrina Múltipla
Medullary Carcinoma of Thyroid
Pheochromocitoma
Multiple Endocrine Neoplasia
topic Carcinoma Medular de Tireóide
Feocromocitoma
Neoplasia Endócrina Múltipla
Medullary Carcinoma of Thyroid
Pheochromocitoma
Multiple Endocrine Neoplasia
description Multiple Endocrine Neoplasia lype ílb (MEN-IIh) is an uncommon syndrome inherited in na autossomal dominant pattern, with 100% penetrance and variable expression. This syndrome is marked by the presence of medullary thyroid carcinoma, pheocromociíoma, and diffuse ganglioneuromatosis of the gastrointestinal tract. Patients have a characteristic fades and marfanoid habitas. The autors report three cases of MEN-IIb, two of them are male, with 15,9 years on average. The first clinical expression in all the cases was the growing cervical mass on the site of the thyroid place and marfanoid habitas. The patients underwent total thyroidectomy with bilateral neck dissection for medullary thyroid carcinoma. One patient had pheochromocitoma fouryears after the diagnosis of the thyroid tumor, and underwent surgical treatment. One patient died 2,5 years after diagnosis, and the others are free of ilness one year after diagnosis.
publishDate 2022
dc.date.none.fl_str_mv 2022-09-21
dc.type.driver.fl_str_mv info:eu-repo/semantics/article
info:eu-repo/semantics/publishedVersion
Relato de Caso
format article
status_str publishedVersion
dc.identifier.uri.fl_str_mv https://rbc.inca.gov.br/index.php/revista/article/view/2776
10.32635/2176-9745.RBC.1999v45n2.2776
url https://rbc.inca.gov.br/index.php/revista/article/view/2776
identifier_str_mv 10.32635/2176-9745.RBC.1999v45n2.2776
dc.language.iso.fl_str_mv por
language por
dc.relation.none.fl_str_mv https://rbc.inca.gov.br/index.php/revista/article/view/2776/1653
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 INCA
publisher.none.fl_str_mv INCA
dc.source.none.fl_str_mv Revista Brasileira de Cancerologia; Vol. 45 No. 2 (1999): Apr./May/June; 47-53
Revista Brasileira de Cancerologia; Vol. 45 Núm. 2 (1999): abr./mayo/jun.; 47-53
Revista Brasileira de Cancerologia; v. 45 n. 2 (1999): abr./maio/jun.; 47-53
2176-9745
reponame:Revista Brasileira de Cancerologia (Online)
instname:Instituto Nacional de Câncer José Alencar Gomes da Silva (INCA)
instacron:INCA
instname_str Instituto Nacional de Câncer José Alencar Gomes da Silva (INCA)
instacron_str INCA
institution INCA
reponame_str Revista Brasileira de Cancerologia (Online)
collection Revista Brasileira de Cancerologia (Online)
repository.name.fl_str_mv Revista Brasileira de Cancerologia (Online) - Instituto Nacional de Câncer José Alencar Gomes da Silva (INCA)
repository.mail.fl_str_mv rbc@inca.gov.br
_version_ 1797042232888393728

Registros relacionados