Peutz-Jeguers syndrome: case report and literature review
Autor(a) principal: | |
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Data de Publicação: | 2012 |
Outros Autores: | , , , , , , |
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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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 |
status_str |
publishedVersion |
dc.identifier.uri.fl_str_mv |
http://old.scielo.br/scielo.php?script=sci_arttext&pid=S2237-93632012000100011 |
url |
http://old.scielo.br/scielo.php?script=sci_arttext&pid=S2237-93632012000100011 |
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 |
eu_rights_str_mv |
openAccess |
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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1752126477004963840 |