Clinical management of hereditary nonpolyposis colon cancer. High-risk clinics and registries.
Early age at onset, multiple primary colon cancers, and other clinical features enable recognition of discrete heritable nonpolyposis colon cancer syndromes, when a pattern involving several close relatives can be demonstrated in specific families. Similar clinical expression can be predicted in as-yet asymptomatic relatives and forms a basis for extraordinary early diagnostic measures, involving frequent endoscopic surveillance starting as early as age 20 to 30 years. When even an early cancer is found, radical measures may be indicated, including total colectomy. The invocation of unusual diagnostic and therapeutic measures is admittedly based on a lack of certainty as to genotypic status and natural history in individual cases; as such, the ongoing zealous search for epithelial and serum markers of risk and early neoplastic change is warranted. Because of the relative infrequency, complexity, and uncertainty surrounding high-risk families, referral to registries that deal on a daily basis with such problems is still indicated.
Lynch
P
P
Section of GI Oncology, University of Texas M.D. Anderson Cancer Center, Houston.
Winn
R J
RJ
D000328:Adult; D000368:Aged; D014408:Biomarkers, Tumor / Q000032:analysis; D002173:Cancer Care Facilities; D003113:Colonoscopy; D003123:Colorectal Neoplasms, Hereditary Nonpolyposis / Q000175:diagnosis* / Q000235:genetics / Q000601:surgery; D005190:Family; D005260:Female; D006782:Hospitals, Special; D006801:Humans; D008297:Male; D008875:Middle Aged; D009955:Ornithine Decarboxylase / Q000032:analysis; D012042:Registries; D012307:Risk Factors
D014408:Biomarkers, Tumor; D009955:Ornithine Decarboxylase