Cáncer colorrectal hereditario no poliposo: tratamiento quirúrgico y análisis de genealogías

ackground: Hereditary nonpolyposis colorectal cancer (HNPCC) accounts for 3 to 5% of all colorectal cancer (CC). It is an autosomal dominant syndrome with 80% of penetrance for this disease. Aim: To analyze the pedigree and surgical treatment of HNPCC. Patients and methods: We retrospectively analyz...

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Autores principales:	Fullerton,Demian A, López K,Francisco, Rahmer O,Alejandro
Lenguaje:	Spanish / Castilian
Publicado:	Sociedad Médica de Santiago 2004
Materias:	Colonic neoplasms Pedigree Rectal neoplasms
Acceso en línea:	http://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872004000500002
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spelling	oai:scielo:S0034-988720040005000022005-11-18Cáncer colorrectal hereditario no poliposo: tratamiento quirúrgico y análisis de genealogíasFullerton,Demian ALópez K,FranciscoRahmer O,Alejandro Colonic neoplasms Pedigree Rectal neoplasms ackground: Hereditary nonpolyposis colorectal cancer (HNPCC) accounts for 3 to 5% of all colorectal cancer (CC). It is an autosomal dominant syndrome with 80% of penetrance for this disease. Aim: To analyze the pedigree and surgical treatment of HNPCC. Patients and methods: We retrospectively analyzed our database of CC selecting patients with HNPCC according to clinical criteria (Amsterdam II). We characterized our patient's pedigrees with telephonic interviews. Results: From 1111 patients operated on with CC we identified 13 (1.17%) with HNPCC. The mean age at diagnosis was 41.6 years (range: 23-75). Sixty two percent presented in International Union Against Cancer (UICC) stages I or II and none in stage IV. Seventy one percent of tumors were proximal to splenic flexure. In 5 patients the diagnosis of HNPCC was made postoperatively, after diagnosis of CC in their relatives. In all but one of the 8 patients with preoperative diagnosis of HNPCC, we performed a total colectomy. From the remaining 6 patients with partial colectomy, 2 developed metachronic CC. Two patients died of cancer. From 101 persons in the 4 families, 25 have developed neoplasia: 18 CC, 3 endometrial cancer and 4 other tumors. Twenty eight relatives were eligible for colonoscopic screening, but only 21% of them have been screened appropriately. Conclusions: Preoperative diagnosis should change the surgical treatment of HNPCC, preventing metachronic disease. Primary colonoscopic screening allowed us to diagnose CC in early stages, nonetheless most of eligible relatives have not followed recommended frequency for colonoscopy (Rev Méd Chile 2004; 132: 539-47).info:eu-repo/semantics/openAccessSociedad Médica de SantiagoRevista médica de Chile v.132 n.5 20042004-05-01text/htmlhttp://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872004000500002es10.4067/S0034-98872004000500002
institution	Scielo Chile
collection	Scielo Chile
language	Spanish / Castilian
topic	Colonic neoplasms Pedigree Rectal neoplasms
spellingShingle	Colonic neoplasms Pedigree Rectal neoplasms Fullerton,Demian A López K,Francisco Rahmer O,Alejandro Cáncer colorrectal hereditario no poliposo: tratamiento quirúrgico y análisis de genealogías
description	ackground: Hereditary nonpolyposis colorectal cancer (HNPCC) accounts for 3 to 5% of all colorectal cancer (CC). It is an autosomal dominant syndrome with 80% of penetrance for this disease. Aim: To analyze the pedigree and surgical treatment of HNPCC. Patients and methods: We retrospectively analyzed our database of CC selecting patients with HNPCC according to clinical criteria (Amsterdam II). We characterized our patient's pedigrees with telephonic interviews. Results: From 1111 patients operated on with CC we identified 13 (1.17%) with HNPCC. The mean age at diagnosis was 41.6 years (range: 23-75). Sixty two percent presented in International Union Against Cancer (UICC) stages I or II and none in stage IV. Seventy one percent of tumors were proximal to splenic flexure. In 5 patients the diagnosis of HNPCC was made postoperatively, after diagnosis of CC in their relatives. In all but one of the 8 patients with preoperative diagnosis of HNPCC, we performed a total colectomy. From the remaining 6 patients with partial colectomy, 2 developed metachronic CC. Two patients died of cancer. From 101 persons in the 4 families, 25 have developed neoplasia: 18 CC, 3 endometrial cancer and 4 other tumors. Twenty eight relatives were eligible for colonoscopic screening, but only 21% of them have been screened appropriately. Conclusions: Preoperative diagnosis should change the surgical treatment of HNPCC, preventing metachronic disease. Primary colonoscopic screening allowed us to diagnose CC in early stages, nonetheless most of eligible relatives have not followed recommended frequency for colonoscopy (Rev Méd Chile 2004; 132: 539-47).
author	Fullerton,Demian A López K,Francisco Rahmer O,Alejandro
author_facet	Fullerton,Demian A López K,Francisco Rahmer O,Alejandro
author_sort	Fullerton,Demian A
title	Cáncer colorrectal hereditario no poliposo: tratamiento quirúrgico y análisis de genealogías
title_short	Cáncer colorrectal hereditario no poliposo: tratamiento quirúrgico y análisis de genealogías
title_full	Cáncer colorrectal hereditario no poliposo: tratamiento quirúrgico y análisis de genealogías
title_fullStr	Cáncer colorrectal hereditario no poliposo: tratamiento quirúrgico y análisis de genealogías
title_full_unstemmed	Cáncer colorrectal hereditario no poliposo: tratamiento quirúrgico y análisis de genealogías
title_sort	cáncer colorrectal hereditario no poliposo: tratamiento quirúrgico y análisis de genealogías
publisher	Sociedad Médica de Santiago
publishDate	2004
url	http://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872004000500002
work_keys_str_mv	AT fullertondemiana cancercolorrectalhereditarionopoliposotratamientoquirurgicoyanalisisdegenealogias AT lopezkfrancisco cancercolorrectalhereditarionopoliposotratamientoquirurgicoyanalisisdegenealogias AT rahmeroalejandro cancercolorrectalhereditarionopoliposotratamientoquirurgicoyanalisisdegenealogias
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Cáncer colorrectal hereditario no poliposo: tratamiento quirúrgico y análisis de genealogías

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