Clinical characteristics and diagnosis of patients with hereditary nonpolyposis colorectal cancer
- PMID: 12532449
- PMCID: PMC4611329
- DOI: 10.3748/wjg.v9.i2.284
Clinical characteristics and diagnosis of patients with hereditary nonpolyposis colorectal cancer
Abstract
Aim: To study the clinical characteristics of hereditary nonpolyposis colorectal cancer (HNPCC) in the Chinese population and discuss the identification and management of the patients with HNPCC.
Methods: A series of 140 patients with colorectal cancers (CRC) and HNPCC associated tumors from 30 families fulfilling the Amsterdam criteria were analyzed.
Results: A total of 118 patients had CRC. Average age at diagnosis of the first CRC was 45.7 years, 56.8 % and 23.4 % of the first CRC were located proximal to the splenic flexure and in the rectum respectively. Twenty-three (19.5 %) had synchronous and metachronous CRC. Twenty-seven patients were found to have extracolonic tumors. Gastric carcinoma was the most common tumor type in our series (44.4 %).
Conclusion: The frequency of HNPCC was 2.6 % in our series of patients. The main features are an excess of early onset with a propensity to involve the proximal colon, and high frequency of multiple foci. Management and surveillance for these patients should be different from sporadic CRC. Contrary to American and European reports, gastric cancer seems more frequent than endometrial cancer in Chinese. It is necessary to formulate a new HNPCC criterion for Chinese patients.
Similar articles
-
[Clinical phenotype of Chinese hereditary nonpolyposis colorectal cancer (HNPCC) families].Zhonghua Zhong Liu Za Zhi. 2006 Jan;28(1):36-8. Zhonghua Zhong Liu Za Zhi. 2006. PMID: 16737618 Chinese.
-
Frequency of extra-colonic tumors in hereditary nonpolyposis colorectal cancer (HNPCC) and familial colorectal cancer (FCC) Brazilian families: An analysis by a Brazilian Hereditary Colorectal Cancer Institutional Registry.Fam Cancer. 2004;3(1):41-7. doi: 10.1023/B:FAME.0000026810.99776.e9. Fam Cancer. 2004. PMID: 15131405
-
Clinical and genetic characteristics of Chinese hereditary nonpolyposis colorectal cancer families.World J Gastroenterol. 2006 Jul 7;12(25):4074-7. doi: 10.3748/wjg.v12.i25.4074. World J Gastroenterol. 2006. PMID: 16810763 Free PMC article.
-
Hereditary nonpolyposis colorectal cancer: diagnostic strategies and their implications.Evid Rep Technol Assess (Full Rep). 2007 May;(150):1-180. Evid Rep Technol Assess (Full Rep). 2007. PMID: 17764220 Free PMC article. Review.
-
What is hereditary nonpolyposis colorectal cancer (HNPCC).Anticancer Res. 1994 Jul-Aug;14(4B):1613-5. Anticancer Res. 1994. PMID: 7979195 Review.
Cited by
-
Profiling of the genetic features of Chinese patients with gastric cancer with HRD germline mutations in a large-scale retrospective study.J Med Genet. 2023 Aug;60(8):760-768. doi: 10.1136/jmg-2022-108816. Epub 2023 Jan 10. J Med Genet. 2023. PMID: 36627197 Free PMC article.
-
Comparative evaluation of immune response after laparoscopical and open total mesorectal excisions with anal sphincter preservation in patients with rectal cancer.World J Gastroenterol. 2003 Dec;9(12):2690-4. doi: 10.3748/wjg.v9.i12.2690. World J Gastroenterol. 2003. PMID: 14669314 Free PMC article.
-
Genetic detection of Chinese hereditary nonpolyposis colorectal cancer.World J Gastroenterol. 2004 Jan 15;10(2):209-13. doi: 10.3748/wjg.v10.i2.209. World J Gastroenterol. 2004. PMID: 14716824 Free PMC article.
-
Molecular analysis: microsatellity instability and loss of heterozygosity of tumor suppressor gene in hereditary non-polyposis colorectal cancers (HNPCC).Bosn J Basic Med Sci. 2009 Feb;9(1):10-8. doi: 10.17305/bjbms.2009.2850. Bosn J Basic Med Sci. 2009. PMID: 19284389 Free PMC article.
-
The tumor spectrum in the Lynch syndrome.Fam Cancer. 2005;4(3):245-8. doi: 10.1007/s10689-004-7994-z. Fam Cancer. 2005. PMID: 16136385 Review.
References
-
- Rodriguez-Bigas MA, Boland CR, Hamilton SR, Henson DE, Jass JR, Khan PM, Lynch H, Perucho M, Smyrk T, Sobin L, et al. A National Cancer Institute Workshop on Hereditary Nonpolyposis Colorectal Cancer Syndrome: meeting highlights and Bethesda guidelines. J Natl Cancer Inst. 1997;89:1758–1762. - PubMed
-
- Debniak T, Kurzawski G, Gorski B, Kladny J, Domagala W, Lubinski J. Value of pedigree/clinical data, immunohistochemistry and microsatellite instability analyses in reducing the cost of determining hMLH1 and hMSH2 gene mutations in patients with colorectal cancer. Eur J Cancer. 2000;36:49–54. - PubMed
-
- Midgley R, Kerr D. Colorectal cancer. Lancet. 1999;353:391–399. - PubMed
Publication types
MeSH terms
LinkOut - more resources
Full Text Sources