THE HAMARTOMATOUS POLYPOSIS SYNDROMES - CLINICAL AND RADIOLOGIC FEATURES

Most radiologists are familiar with the clinical and radiologic featur es of the familial adenomatous polyposis syndromes [1], The hamartomat ous polyposis syndromes occur less frequently, however, and their radi ologic and clinical manifestations are not as well known. This group o f syndromes includes Peutz-Jeghers, multiple hamartoma, juvenile polyp osis, Cronkhite-Canada, and Bannayan-Riley-Ruvalcaba. The predominant gastrointestinal lesion in these diseases is some form of hamartomatou s polyp, The term hamartoma implies a nonneoplastic tumor or tumorlike condition composed of tissue elements normally present in the particu lar area [2], In many of these syndromes, it is now recognized that ha martomatous polyps of the gastrointestinal tract coexist with adenomas and that adenomas may develop within hamartomatous polyps. Either sit uation may contribute to the frequent association of alimentary tract adenocarcinoma that occurs in most of these syndromes, Various types o f benign mucocutaneous lesions are common and often lead to the correc t diagnosis, Of greater importance is the frequent occurrence of other extraintestinal manifestations, including several forms of malignant disease, Because of this frequent association with both gastrointestin al and nongastrointestinal malignant tumors, early and accurate diagno sis of these syndromes is essential, Meticulously performed double con trast studies are the preferred radiologic procedures for the diagnosi s of gastrointestinal polyps in all of these diseases.