By Gary M. White, MD
The Gardner syndrome (GS) is a symptom complex characterized by intestinal polyposis, mandibular, maxillary and long bone osteomas, fibromas, lipomas, dental abnormalities (mandibular cysts, impacted teeth, and supernumerary teeth) and epidermal inclusion cysts.
- Gardner syndrome shares all the features of colonic polyposis or familial adenomatous polyposis and is inherited AD.
- These patients have a high incidence of desmoid tumors.
- Other findings include neoplasms of the thyroid, adrenals, biliary tree, and liver and congenital hypertrophy of the retinal pigment epithelium which appears as single or multiple pigmented ovoid lesions occurring unilaterally or bilaterally and detectable by routine funduscopic examination.
- Teeth abnormalities include multiple impacted or unerupted teeth, supernumerary teeth, hypodontia, compound odontomas, and dentigerous cyst.
It is thought that Gardner syndrome is a variant of familial adenomatous polyposis. The genetic defect of both diseases is the APC (adenomatous polyposis coli) gene found on chromosome 5 which is a classic tumor-suppressor gene.
Given that the intestinal polyps have nearly 100% malignant potential if untreated, the important management issues are handled by gastroenterology. Cutaneous cysts may be surgically excised.
Journal of the American Academy of Dermatology Volume 68, Issue 2, Pages 189.e1–189.e21, February 2013
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