Abstract

Familial Adenomatous Polyposis (FAP) is a rare and autosomal dominantly inherited disorder characterized by the development of hundreds to thousands of colorectal polyps. Korean Polyposis Registry was established in July, 1990 for early detection and management of the FAP patient. Recently, we have experienced in our institution a case of the FAP family kindred living in Jeju Island of Korea. Their relatives have been managed for the past 20 years and are listed in the Korean polyposis registry. Pathologic diagnosis of our proband was stage III (T3N1M0) rectal cancer with thousands of colonic polyps. Intrafamilial strife and psychologic distress was significant due to the late detection and progression to rectal cancer. Therefore, we reviewed our case of the FAP family with literature regarding the psychologic distress and the role of the regional registry.

• Keywords: Familial adenomatous polyposis;Pedigree;Psychologic distress;Registry