HEREDITARY NONPOLYPOSIS COLORECTAL-CANCER - CLINICAL-FEATURES AND SURVIVAL - RESULTS FROM THE DANISH HNPCC REGISTER

Background: Hereditary non-polyposis colorectal cancer (HNPCC) is a do minantly inherited syndrome characterized by the development of colore ctal cancer (CRC) and other carcinomas. Our aim was to evaluate tumour parameters and survival in HNPCC. Methods: One hundred and eight Dani sh HNPCC patients were compared with 870 patients with sporadic colore ctal cancer. Results: The median age at CRC diagnosis was 41 years in the HNPCC group. HNPCC patients had significantly more carcinomas loca ted to the right colon (68% against 49% in controls), more synchronous tumours (7% versus 1%), more metachronous CRC after 10 years (29% ver sus 5%), more localized carcinomas (62% versus 39%), and significantly higher crude cumulative 5-year survival (56% versus 30%). Conclusions : CRC in HNPCC behaves differently compared to sporadic cases concerni ng age of onset, frequency of multiple lesions, and location. The meta static tendency is less than in sporadic CRC and the survival is bette r.