Familial polyposis coli.

Abstract

Familial polyposis coli is a syndrome of autosomal dominant inheritance that ultimately causes colonic cancer if untreated. Diagnosis at the presymptomatic stage is imperative. Patients with the disease, as well as family members at risk, need intense support, education, surveillance and follow-up. Benign and malignant extracolonic manifestations, such as osteomas, desmoid tumors, medulloblastomas and glioblastomas, may appear even before the polyposis is clinically evident. When symptoms of the polyps are present, two-thirds of the patients already have colonic cancer.