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Every 1-2 years

* Individuals with a family history of these neoplasms.

Modified from International Collaborative Group on Hereditary Non-Polyposis Colorectal Cancer (2003). Used with permission.

* Individuals with a family history of these neoplasms.

Modified from International Collaborative Group on Hereditary Non-Polyposis Colorectal Cancer (2003). Used with permission.

patients still have the same risk of developing cancer as the general population.

At M. D. Anderson, colorectal cancers from individuals suspected of having HNPCC are tested for hMLHl, hMSH2, and hMSH6 protein expression by immunohistochemical analysis. We used to test these tumors for MSI since MSI is present in more than 85% of colorectal cancers from HNPCC patients. However, a recently published study evaluating hMLHl and hMSH2 protein expression reported a 92% sensitivity and a l00% positive predictive value for MSI with absence of hMLHl and hMSH2 protein expression (Lindor et al, 2002). Therefore, immunohisto-chemical analysis appears to be a reliable method to screen for MSI, and in fact, the absence of protein expression serves as a guide as to which gene to test for mutation. Nevertheless, we still use MSI testing in selected situations.

Treatment for Patients with HNPCC

Because of the increased incidence of synchronous and metachronous colorectal neoplasms in patients with HNPCC, the treatment of choice for patients with HNPCC is a total abdominal colectomy with ileorectal anastomosis. However, no prospective studies show a survival advantage for this treatment over segmental resection. Therefore, even though the treatment of choice is a total abdominal colectomy with ileorectal anastomosis, the procedure should be individualized. In women who are post-menopausal or who have completed their family, consideration should be given to prophylactic hysterectomy and bilateral salpingo-oophorectomy. However, there are no data to suggest a survival benefit from the latter procedure. In HNPCC patients treated with less than an abdominal colec-tomy, surveillance should be the mainstay of treatment, and inclusion in

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