[Familial juvenile polyposis with predominant stomach involvement]

Juvenile Polyposis (JP) is a rare disease that may be found anywhere within the gastrointestinal tract, almost most cases so far reported have involved the colon. It is a precancerous condition, with the subsequently developing carcinomas so far also being found almost exclusively in the colon. A familial form is found in 20 to 50% of the cases. The present paper describes a family in whom three members of the second generation developed massive JP in the stomach requiring partial resection of the stomach or gastrectomy. Three members of the first generation died of carcinoma of the stomach and a forth of carcinoma of the colon. A male member of the second generation was treated at the age of 38 years for a carcinoma of the colon; 16 years later, he underwent resection of the stomach for juvenile polyposis and the histological work-up of the surgical specimen revealed in addition, areas of dysplasia and early carcinomas restricted to the mucosa.