EPIDEMIOLOGY, SYMPTOMS, MORPHOLOGY AND TH ERAPY OF THE CRONKHITE-CANADA-SYNDROME - BASED ON A CASE-REPORT AND THE LITERATURE

Juvenile polyposis was first described by Cronkhite and Canada in 1955 . This disease is characterized by juvenile intestinal polyps and ecto dermal abnormalities. The etiology of Cronkhite-Canada syndrome (CCS), however, is still not well understood. Interestingly, among patients with CCS a significant correlation (16.5 %) with intestinal carcinomas has been observed. Thus, malignant transformation and/or genetic pred isposition may be involved in the initiation of the disease. In the fo llowing, epidemiology, symptoms, morphology and therapy of CCS are dis cussed. Our examinations are based on studies reported in the literatu re and on a case report of a female patient who developed a colon carc inoma 2 years after initial diagnosis of CCS.