Desmoid tumor in patients with familial adenomatous polyposis

Detalhes bibliográficos
Autor(a) principal: Leal,Raquel Franco
Data de Publicação: 2010
Outros Autores: Silva,Patricia V. V. Tapia, Ayrizono,Maria de Lourdes Setsuko, Fagundes,João José, Amstalden,Eliane M. Ingrid, Coy,Cláudio Saddy Rodrigues
Tipo de documento: Artigo
Idioma: eng
Título da fonte: Arquivos de gastroenterologia (Online)
Texto Completo: http://old.scielo.br/scielo.php?script=sci_arttext&pid=S0004-28032010000400010
Resumo: CONTEXT: Desmoid tumors constitute one of the most important extraintestinal manifestations of familial adenomatous polyposis. The development of desmoids is responsible for increasing morbidity and mortality rates in cases of familial adenomatous polyposis. OBJECTIVES: To evaluate the occurrence of desmoid tumors in familial adenomatous polyposis cases following prophylactic colectomy and to present patient outcome. METHODS: Between 1984 and 2008, 68 patients underwent colectomy for familial adenomatous polyposis at the School of Medical Sciences Teaching Hospital, University of Campinas, SP, Brazil. Desmoid tumors were found in nine (13.2%) of these patients, who were studied retrospectively by consulting their medical charts with respect to clinical and surgical data. RESULTS: Of nine patients, seven (77.8%) were submitted to laparotomy for tumor resection. Median age at the time of surgery was 33.9 years (range 22-51 years). Desmoid tumors were found in the abdominal wall in 3/9 cases (33.3%) and in an intra-abdominal site in the remaining six cases (66.7%). Median time elapsed between ileal pouch-anal anastomosis and diagnosis of desmoid tumor was 37.5 months (range 14-60 months), while the median time between colectomy with ileorectal anastomosis and diagnosis was 63.7 months (range 25-116 months). In 6/9 (66.7%) patients with desmoid tumors, the disease was either under control or there was no evidence of tumor recurrence at a follow-up visit made a mean of 63.1 months later (range 12-240 months). CONCLUSIONS: Desmoid tumors were found in 13.2% of cases of familial adenomatous polyposis following colectomy; therefore, familial adenomatous polyposis patients should be followed-up and surveillance should include abdominal examination to detect signs and symptoms. Treatment options include surgery and clinical management with antiestrogens, antiinflammatory drugs or chemotherapy.
id IBEPEGE-1_04e7e47e8d78ccd1d7463fea2a2ba773
oai_identifier_str oai:scielo:S0004-28032010000400010
network_acronym_str IBEPEGE-1
network_name_str Arquivos de gastroenterologia (Online)
repository_id_str
spelling Desmoid tumor in patients with familial adenomatous polyposisFibromatosis, abdominalAdenomatous polyposis coliAnastomosis, surgicalColonic pouchesCONTEXT: Desmoid tumors constitute one of the most important extraintestinal manifestations of familial adenomatous polyposis. The development of desmoids is responsible for increasing morbidity and mortality rates in cases of familial adenomatous polyposis. OBJECTIVES: To evaluate the occurrence of desmoid tumors in familial adenomatous polyposis cases following prophylactic colectomy and to present patient outcome. METHODS: Between 1984 and 2008, 68 patients underwent colectomy for familial adenomatous polyposis at the School of Medical Sciences Teaching Hospital, University of Campinas, SP, Brazil. Desmoid tumors were found in nine (13.2%) of these patients, who were studied retrospectively by consulting their medical charts with respect to clinical and surgical data. RESULTS: Of nine patients, seven (77.8%) were submitted to laparotomy for tumor resection. Median age at the time of surgery was 33.9 years (range 22-51 years). Desmoid tumors were found in the abdominal wall in 3/9 cases (33.3%) and in an intra-abdominal site in the remaining six cases (66.7%). Median time elapsed between ileal pouch-anal anastomosis and diagnosis of desmoid tumor was 37.5 months (range 14-60 months), while the median time between colectomy with ileorectal anastomosis and diagnosis was 63.7 months (range 25-116 months). In 6/9 (66.7%) patients with desmoid tumors, the disease was either under control or there was no evidence of tumor recurrence at a follow-up visit made a mean of 63.1 months later (range 12-240 months). CONCLUSIONS: Desmoid tumors were found in 13.2% of cases of familial adenomatous polyposis following colectomy; therefore, familial adenomatous polyposis patients should be followed-up and surveillance should include abdominal examination to detect signs and symptoms. Treatment options include surgery and clinical management with antiestrogens, antiinflammatory drugs or chemotherapy.Instituto Brasileiro de Estudos e Pesquisas de Gastroenterologia e Outras Especialidades - IBEPEGE. 2010-12-01info:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersiontext/htmlhttp://old.scielo.br/scielo.php?script=sci_arttext&pid=S0004-28032010000400010Arquivos de Gastroenterologia v.47 n.4 2010reponame:Arquivos de gastroenterologia (Online)instname:Instituto Brasileiro de Estudos e Pesquisas de Gastroenterologiainstacron:IBEPEGE10.1590/S0004-28032010000400010info:eu-repo/semantics/openAccessLeal,Raquel FrancoSilva,Patricia V. V. TapiaAyrizono,Maria de Lourdes SetsukoFagundes,João JoséAmstalden,Eliane M. IngridCoy,Cláudio Saddy Rodrigueseng2011-01-03T00:00:00Zoai:scielo:S0004-28032010000400010Revistahttp://www.scielo.br/aghttps://old.scielo.br/oai/scielo-oai.php||secretariaarqgastr@hospitaligesp.com.br1678-42190004-2803opendoar:2011-01-03T00:00Arquivos de gastroenterologia (Online) - Instituto Brasileiro de Estudos e Pesquisas de Gastroenterologiafalse
dc.title.none.fl_str_mv Desmoid tumor in patients with familial adenomatous polyposis
title Desmoid tumor in patients with familial adenomatous polyposis
spellingShingle Desmoid tumor in patients with familial adenomatous polyposis
Leal,Raquel Franco
Fibromatosis, abdominal
Adenomatous polyposis coli
Anastomosis, surgical
Colonic pouches
title_short Desmoid tumor in patients with familial adenomatous polyposis
title_full Desmoid tumor in patients with familial adenomatous polyposis
title_fullStr Desmoid tumor in patients with familial adenomatous polyposis
title_full_unstemmed Desmoid tumor in patients with familial adenomatous polyposis
title_sort Desmoid tumor in patients with familial adenomatous polyposis
author Leal,Raquel Franco
author_facet Leal,Raquel Franco
Silva,Patricia V. V. Tapia
Ayrizono,Maria de Lourdes Setsuko
Fagundes,João José
Amstalden,Eliane M. Ingrid
Coy,Cláudio Saddy Rodrigues
author_role author
author2 Silva,Patricia V. V. Tapia
Ayrizono,Maria de Lourdes Setsuko
Fagundes,João José
Amstalden,Eliane M. Ingrid
Coy,Cláudio Saddy Rodrigues
author2_role author
author
author
author
author
dc.contributor.author.fl_str_mv Leal,Raquel Franco
Silva,Patricia V. V. Tapia
Ayrizono,Maria de Lourdes Setsuko
Fagundes,João José
Amstalden,Eliane M. Ingrid
Coy,Cláudio Saddy Rodrigues
dc.subject.por.fl_str_mv Fibromatosis, abdominal
Adenomatous polyposis coli
Anastomosis, surgical
Colonic pouches
topic Fibromatosis, abdominal
Adenomatous polyposis coli
Anastomosis, surgical
Colonic pouches
description CONTEXT: Desmoid tumors constitute one of the most important extraintestinal manifestations of familial adenomatous polyposis. The development of desmoids is responsible for increasing morbidity and mortality rates in cases of familial adenomatous polyposis. OBJECTIVES: To evaluate the occurrence of desmoid tumors in familial adenomatous polyposis cases following prophylactic colectomy and to present patient outcome. METHODS: Between 1984 and 2008, 68 patients underwent colectomy for familial adenomatous polyposis at the School of Medical Sciences Teaching Hospital, University of Campinas, SP, Brazil. Desmoid tumors were found in nine (13.2%) of these patients, who were studied retrospectively by consulting their medical charts with respect to clinical and surgical data. RESULTS: Of nine patients, seven (77.8%) were submitted to laparotomy for tumor resection. Median age at the time of surgery was 33.9 years (range 22-51 years). Desmoid tumors were found in the abdominal wall in 3/9 cases (33.3%) and in an intra-abdominal site in the remaining six cases (66.7%). Median time elapsed between ileal pouch-anal anastomosis and diagnosis of desmoid tumor was 37.5 months (range 14-60 months), while the median time between colectomy with ileorectal anastomosis and diagnosis was 63.7 months (range 25-116 months). In 6/9 (66.7%) patients with desmoid tumors, the disease was either under control or there was no evidence of tumor recurrence at a follow-up visit made a mean of 63.1 months later (range 12-240 months). CONCLUSIONS: Desmoid tumors were found in 13.2% of cases of familial adenomatous polyposis following colectomy; therefore, familial adenomatous polyposis patients should be followed-up and surveillance should include abdominal examination to detect signs and symptoms. Treatment options include surgery and clinical management with antiestrogens, antiinflammatory drugs or chemotherapy.
publishDate 2010
dc.date.none.fl_str_mv 2010-12-01
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=S0004-28032010000400010
url http://old.scielo.br/scielo.php?script=sci_arttext&pid=S0004-28032010000400010
dc.language.iso.fl_str_mv eng
language eng
dc.relation.none.fl_str_mv 10.1590/S0004-28032010000400010
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 Instituto Brasileiro de Estudos e Pesquisas de Gastroenterologia e Outras Especialidades - IBEPEGE.
publisher.none.fl_str_mv Instituto Brasileiro de Estudos e Pesquisas de Gastroenterologia e Outras Especialidades - IBEPEGE.
dc.source.none.fl_str_mv Arquivos de Gastroenterologia v.47 n.4 2010
reponame:Arquivos de gastroenterologia (Online)
instname:Instituto Brasileiro de Estudos e Pesquisas de Gastroenterologia
instacron:IBEPEGE
instname_str Instituto Brasileiro de Estudos e Pesquisas de Gastroenterologia
instacron_str IBEPEGE
institution IBEPEGE
reponame_str Arquivos de gastroenterologia (Online)
collection Arquivos de gastroenterologia (Online)
repository.name.fl_str_mv Arquivos de gastroenterologia (Online) - Instituto Brasileiro de Estudos e Pesquisas de Gastroenterologia
repository.mail.fl_str_mv ||secretariaarqgastr@hospitaligesp.com.br
_version_ 1754193345695973376