Gardner's Syndrome in a 75-Year-Old Woman

Abstract

Familial adenomatous polyposis (FAP) is an inherited disease characterized by the presence of multiple polyps in the colon or other areas of the gastrointestinal tract. In Gardner's syndrome, which is considered by some as a variant of FAP, polyps in the colon present with extracolonic manifestations, such as osteomas, lipomas, fibromas or other soft tissue tumors. In 100% of all untreated patients, cancer of the large intestine develops before the age of 40. Subjects with this disorder usually die from complications related to this and other tumors. Older patients surviving this disease only did so after total colectomy was performed.
We describe a 75-year-old patient with the typical lesions of Gardner's syndrome who survived without treatment for the disease for more than 40 years after diagnosis.