대한대장항문학회

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