Gardner's syndrome

The Gardner 's syndrome, named after the American geneticist Eldon John Gardner (* 1909 in Utah, † 1989), is a rare hereditary tumor disease, polyposis coli protein is a mutation of the Adenomatous basis. It occurs in about one in every 10,000 births and is inherited as an autosomal dominant trait.

The syndrome is described as phenotypic variant of familial adenomatous polyposis (FAP) (i.e., there is a smooth transition ). In addition to adenomas of the colon occur benign tumors of the bone (eg, osteomas ), the skin, subcutaneous tissue and connective tissue ( eg, atheroma, lipoma, fibroma, leiomyoma ). Other features that are associated with Gardner 's syndrome, Hyperdontie are ( tooth number ), colonic and gastric polyps and epidermoid cysts.

Therapy

Since the Gardner syndrome is just as familial adenomatous polyposis an obligatory precancerous lesion (100 % probability of occurrence of colorectal cancer ), treatment is early total colectomy.