FAMILIAL JUVENILE POLYPOSIS WITH ADENOMATOUS-CARCINOMATOUS CHANGE

A family tree of 118 members with five members found to have juvenile polyposis-adenomatous change and four juvenile polyposis-adenomatous-c arcinomatous change is presented. All the patients developed bleeding per rectum between 10 and 17 years of age. Four members died of coloni c malignancy between 30 and 55 years of age. Colonoscopy in five livin g members revealed typical juvenile polyps throughout the whole length of the colon and atypical large lobulated polyps containing adenomato us change in juvenile polyps in the rectosigmoid area. An autosomal do minant mode of transmission was evident on analysis of the pedigree. G astric hyperplastic polyps were present in three of the five living me mbers. Familial juvenile polyposis may have the potential to progress into adenoma-carcinoma sequence and is not always a benign disorder. C olonoscopic surveillance should be done to detect adenomatous change i f any member of the juvenile polyposis family develops colonic maligna ncy.