Peutz-Jeguers syndrome: case report and literature review

Detalhes bibliográficos
Autor(a) principal: Torres Neto,Juvenal da Rocha
Data de Publicação: 2012
Outros Autores: Santiago,Rodrigo R., Prudente,Ana Carolina Lisboa, Mariano,Dan Rodrigues, Torres,Felipe Augusto do Prado, Torres,Júlio Augusto do Prado, Ramos,Fernanda Mendonça, Santana,Raquel Matos de
Tipo de documento: Artigo
Idioma: eng
Título da fonte: Journal of Coloproctology (Rio de Janeiro. Online)
Texto Completo: http://old.scielo.br/scielo.php?script=sci_arttext&pid=S2237-93632012000100011
Resumo: The Peutz-Jeghers syndrome is a rare disease characterized by the presence of mucocutaneous melanic pigmentation of the lips, oral mucosa and perioral region, associated with hamartomatous intestinal polyposis. Malignization of the polyps and association with other types of cancer are also usual. Case report: 32-year-old patient, female, white, who had an intestinal occlusion by invagination, discovered during laparotomy, when an intestinal tumor was found as well. The material was sent to anotomopathological analysis. However, the results did not allow to identify the tumor nature due to tumor necrosis. Then, the patient was sent to our service because of the intestinal polyps, and during the interview, the characteristic melanic pigmentation was observed. Videocolonoscopy was performed, with excision of two rectal polyps, identified in the anatomopathological exam as hamartomatous polyps. The patient reported anal imperforation at birth, just like her brother. He had unexplained death. The authors found no correlation of the Peutz-Jeghers syndrome with anal imperforation in the literature and asked the patient if her brother also had the syndrome.
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spelling Peutz-Jeguers syndrome: case report and literature reviewPeutz-Jeghers syndromepolyposishamartomatous intestinalanus, imperforatehyperpigmentationThe Peutz-Jeghers syndrome is a rare disease characterized by the presence of mucocutaneous melanic pigmentation of the lips, oral mucosa and perioral region, associated with hamartomatous intestinal polyposis. Malignization of the polyps and association with other types of cancer are also usual. Case report: 32-year-old patient, female, white, who had an intestinal occlusion by invagination, discovered during laparotomy, when an intestinal tumor was found as well. The material was sent to anotomopathological analysis. However, the results did not allow to identify the tumor nature due to tumor necrosis. Then, the patient was sent to our service because of the intestinal polyps, and during the interview, the characteristic melanic pigmentation was observed. Videocolonoscopy was performed, with excision of two rectal polyps, identified in the anatomopathological exam as hamartomatous polyps. The patient reported anal imperforation at birth, just like her brother. He had unexplained death. The authors found no correlation of the Peutz-Jeghers syndrome with anal imperforation in the literature and asked the patient if her brother also had the syndrome.Sociedade Brasileira de Coloproctologia2012-03-01info:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersiontext/htmlhttp://old.scielo.br/scielo.php?script=sci_arttext&pid=S2237-93632012000100011Journal of Coloproctology (Rio de Janeiro) v.32 n.1 2012reponame:Journal of Coloproctology (Rio de Janeiro. Online)instname:Sociedade Brasileira de Coloproctologia (SBCP)instacron:SBCP10.1590/S2237-93632012000100011info:eu-repo/semantics/openAccessTorres Neto,Juvenal da RochaSantiago,Rodrigo R.Prudente,Ana Carolina LisboaMariano,Dan RodriguesTorres,Felipe Augusto do PradoTorres,Júlio Augusto do PradoRamos,Fernanda MendonçaSantana,Raquel Matos deeng2012-06-29T00:00:00Zoai:scielo:S2237-93632012000100011Revistahttp://www.scielo.br/scielo.php?script=sci_serial&pid=2237-9363&lng=pt&nrm=isohttps://old.scielo.br/oai/scielo-oai.php||sbcp@sbcp.org.br2317-64232237-9363opendoar:2012-06-29T00:00Journal of Coloproctology (Rio de Janeiro. Online) - Sociedade Brasileira de Coloproctologia (SBCP)false
dc.title.none.fl_str_mv Peutz-Jeguers syndrome: case report and literature review
title Peutz-Jeguers syndrome: case report and literature review
spellingShingle Peutz-Jeguers syndrome: case report and literature review
Torres Neto,Juvenal da Rocha
Peutz-Jeghers syndrome
polyposis
hamartomatous intestinal
anus, imperforate
hyperpigmentation
title_short Peutz-Jeguers syndrome: case report and literature review
title_full Peutz-Jeguers syndrome: case report and literature review
title_fullStr Peutz-Jeguers syndrome: case report and literature review
title_full_unstemmed Peutz-Jeguers syndrome: case report and literature review
title_sort Peutz-Jeguers syndrome: case report and literature review
author Torres Neto,Juvenal da Rocha
author_facet Torres Neto,Juvenal da Rocha
Santiago,Rodrigo R.
Prudente,Ana Carolina Lisboa
Mariano,Dan Rodrigues
Torres,Felipe Augusto do Prado
Torres,Júlio Augusto do Prado
Ramos,Fernanda Mendonça
Santana,Raquel Matos de
author_role author
author2 Santiago,Rodrigo R.
Prudente,Ana Carolina Lisboa
Mariano,Dan Rodrigues
Torres,Felipe Augusto do Prado
Torres,Júlio Augusto do Prado
Ramos,Fernanda Mendonça
Santana,Raquel Matos de
author2_role author
author
author
author
author
author
author
dc.contributor.author.fl_str_mv Torres Neto,Juvenal da Rocha
Santiago,Rodrigo R.
Prudente,Ana Carolina Lisboa
Mariano,Dan Rodrigues
Torres,Felipe Augusto do Prado
Torres,Júlio Augusto do Prado
Ramos,Fernanda Mendonça
Santana,Raquel Matos de
dc.subject.por.fl_str_mv Peutz-Jeghers syndrome
polyposis
hamartomatous intestinal
anus, imperforate
hyperpigmentation
topic Peutz-Jeghers syndrome
polyposis
hamartomatous intestinal
anus, imperforate
hyperpigmentation
description The Peutz-Jeghers syndrome is a rare disease characterized by the presence of mucocutaneous melanic pigmentation of the lips, oral mucosa and perioral region, associated with hamartomatous intestinal polyposis. Malignization of the polyps and association with other types of cancer are also usual. Case report: 32-year-old patient, female, white, who had an intestinal occlusion by invagination, discovered during laparotomy, when an intestinal tumor was found as well. The material was sent to anotomopathological analysis. However, the results did not allow to identify the tumor nature due to tumor necrosis. Then, the patient was sent to our service because of the intestinal polyps, and during the interview, the characteristic melanic pigmentation was observed. Videocolonoscopy was performed, with excision of two rectal polyps, identified in the anatomopathological exam as hamartomatous polyps. The patient reported anal imperforation at birth, just like her brother. He had unexplained death. The authors found no correlation of the Peutz-Jeghers syndrome with anal imperforation in the literature and asked the patient if her brother also had the syndrome.
publishDate 2012
dc.date.none.fl_str_mv 2012-03-01
dc.type.driver.fl_str_mv info:eu-repo/semantics/article
dc.type.status.fl_str_mv info:eu-repo/semantics/publishedVersion
format article
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dc.language.iso.fl_str_mv eng
language eng
dc.relation.none.fl_str_mv 10.1590/S2237-93632012000100011
dc.rights.driver.fl_str_mv info:eu-repo/semantics/openAccess
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dc.format.none.fl_str_mv text/html
dc.publisher.none.fl_str_mv Sociedade Brasileira de Coloproctologia
publisher.none.fl_str_mv Sociedade Brasileira de Coloproctologia
dc.source.none.fl_str_mv Journal of Coloproctology (Rio de Janeiro) v.32 n.1 2012
reponame:Journal of Coloproctology (Rio de Janeiro. Online)
instname:Sociedade Brasileira de Coloproctologia (SBCP)
instacron:SBCP
instname_str Sociedade Brasileira de Coloproctologia (SBCP)
instacron_str SBCP
institution SBCP
reponame_str Journal of Coloproctology (Rio de Janeiro. Online)
collection Journal of Coloproctology (Rio de Janeiro. Online)
repository.name.fl_str_mv Journal of Coloproctology (Rio de Janeiro. Online) - Sociedade Brasileira de Coloproctologia (SBCP)
repository.mail.fl_str_mv ||sbcp@sbcp.org.br
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