A subtype of familial adenomatous polyposis with extracolonic findings including desmoid tumors, congenital hypertrophy retinal pigment, dental abnormalities, osteomas of bones, jaw cysts and epidermoid cysts.
Gardner syndrome is a rare, inherited disorder characterized by multiple polyps) in the colon, often 1,000 or more), extra teeth , bony tumors of the skull (osteomas), and fatty cysts and/or fibrous tumors in the skin (fibromas or epithelial cysts).
Gardner syndrome is a variant of familial adenomatous polyposis, a rare group of disorders characterized by the growth of multiple polyps in the colon.
Autosomal dominant disorder.
Associated with extensive adenomatous polyps of the colon and rectum and other areas of the gastrointestinal tract.
Polyps associated with high transition rate to malignancy occurring by age 35-40 years.
Incidence of thyroid cancer is increased, especially in women.
Skin lesions include: large epidermis cysts, fibromas, lipomas, leiomyoma, trichoepitheliomas, and neurofibromas.
Approximately 50% of patients experience a membranous bone osteomas of the face or skull.
Gardner syndrome is characterized by FAP (familial adenomatous polyps), osseous and soft tissue tumors, retinal pigment epithelium hypertrophy and impacted teeth.