A case of Gardner's syndrome associated with adrenal adenoma / 대한내과학회지

ABSTRACT

Gardners syndrome is a single gene disorder with variable manifestations associated with the adenomatous polyposis coli (APC) gene; including gastrointestinal polyposis and osteomas associated with a variety of benign soft tissue tumors and other extraintestinal manifestations. Infrequently associated tumors include papillary carcinoma of the thyroid, biliary neoplasia and adrenal neoplasia, both benign and malignant. It is classified as a variant of classic familial adenomatous polyposis (FAP) and is an autosomal dominant disease. The incidence of incidentaloma in FAP patients is known to be higher than general population. The occurrence of an adrenal carcinoma could affect a patient's prognosis with Gardners syndrome. Increased awareness of this lesion is important in patients with Gardners syndrome who have extended lifespan after prophylactic colectomy. We report a 37 year old case of Gardners syndrome associate with adrenal adenoma.