Hereditary Nonpolyposis Colorectal Cancer

ABSTRACT

Hereditary nonpolyposis colorectal cancer(HNPCC) is an autosomal dominantly inherited disease associated with a marked increase in cancer susceptibility, especially cancer of the colorectum. The frequency of HNPCC in the general population is yet to be determined, but HNPCC may account for as much as 2% to 5% of colorectal cancer, Colorectal cancer in HNPCC differs from sporadic colorectal cancer by an early age of cancer onset, proximal predominance of colorectal cancer, an excess of synchronous and metachronous colorectal cancer, and excess extra-colonic cancers. We have found 5 HNPCC families since 1992 when we reported first HNPCC family (KHU-Hl) In order to register the patients of HNPCC and to review the clinicopathologic feature and appropriate management, we have analysed 5 HNPCC families. Five HNPCC families included 16 colorectal cancer patients(14 males and 2 females). The average age of first diagnosis was 39. Among 16 patients, 8 patient were operated at the KyungHee University hospital and their operative and pathologic records were available. Two synchronous and seven metachronous cancers were founded, so that eight patients had 15 colorectal cancer lesions. Ten cancers were located proximal to splenic flexure and five were distal. Partial resection of colon was performed in seven cases except one when the first diagnosis was made and recurrence was founded in 5 patients. Recurrence was treated by total colectomy in 3 cases and subtotal colectomy in two. In conclusion, we re-confirmed that HNPCC patient should be treated by no less than a subtotal colectomy because of high multiplicity and high recurrence rate of partial resection.