Babs G. Taal

ENETS Consensus Guidelines for the Management of Patients with Digestive Neuroendocrine Neoplasms of the Digestive System : Well-Differentiated Pancreatic Non-Functioning Tumors

New epidemiological data come from a study performed in Argentina These data are similar to the SEER data, where g-NENs were found to represent 8.7% of all enteric NENs [3] , and quite similar to a recent prospective Austrian study by Niederle et al. [4] , where g-NENs represented 5.6% of all digestive NENs. The proportions of g-NENs with respect to the overall NEN rates do vary, however; g-NENs represented 23% of all NENs in the Austrian study compared to 6% in the SEER data, 5% in a Canadian study (Ontario) and 7.4% in a Taiwanese study These differences underline the need for multicenter prospective studies with long-term analysis to better describe the European epidemiology of these tumors.

PURPOSE: The bowel and sexual function of colorectal cancer patients undergoing either sphincter-saving or sphincter-sacrificing surgical procedures may be impaired. A legitimate question is how these different surgical techniques affect the patients' quality of life. METHODS: Seventeen studies were identified that compared at least one of four aspects of patient functioning (i.e., physical, psychologic, social, and sexual) between stoma patients and nonstoma patients. RESULTS: Although the literature does not yield entirely consistent findings, some long-term effects of surgery can be identified: 1) both patient groups are troubled by frequent or irregular bowel movements and diarrhea; 2) stoma patients report higher levels of psychologic distress than do nonstoma patients; 3) although both stoma patients and nonstoma patients report restrictions in their level of social functioning, such problems are more prevalent among patients with a colostomy; 4) sexual functioning of male and female stoma patients is consistently more impaired than that of male and female patients with intact sphincters. Results of the current review were compared with those of other, related areas. CONCLUSIONS: Although nonstoma patients generally fare better than do stoma patients, they also suffer from physical impairments induced by sphincter-saving procedures (e.g., impaired bowel and sexual function). These impairments may become more prevalent as ultralow anastomosis is more frequently applied, resulting in bowel and sexual dysfunction and related psychologic distress. Well-designed studies are needed that examine whether quality-of-life benefits are to be gained by use of ultralow anastomosis compared with colostomy.

PURPOSE: Hereditary nonpolyposis colorectal cancer (HNPCC) is an autosomal dominant disease characterized by the clustering of colorectal cancer, endometrial cancer, and various other cancers. The disease is caused by mutations in DNA-mismatch-repair (MMR) genes, most frequently in MLH1, MSH2, and MSH6. The aims of the present study were to compare the risk of developing colorectal, endometrial, and other cancers between families with the various MMR-gene mutations. PATIENTS AND METHODS: Clinical and pathologic data were collected from 138 families with HNPCC. Mutation analyses were performed for all families. Survival analysis was used to calculate the cumulative risk of developing cancer in the various subsets of relatives. RESULTS: Mutations were identified in 79 families: 34 in MLH1, 40 in MSH2, and five in MSH6. The lifetime risk of developing cancer at any site was significantly higher for MSH2 mutation carriers than for MLH1 mutation carriers (P < .01). The risk of developing colorectal or endometrial cancer was higher in MSH2 mutation carriers than in MLH1 mutation carriers, but the difference was not significant (P = .13 and P = .057, respectively). MSH2 mutation carriers were found to have a significantly higher risk of developing cancer of the urinary tract (P < .05). The risk of developing cancer of the ovaries, stomach, and brain was also higher in the MSH2 mutation carriers than in the MLH1 mutation carriers, but the difference was not statistically significant. CONCLUSION: Pending large prospective studies, the extension of the current surveillance program in MSH2 mutation carriers with the inclusion of the urinary tract should be considered.