Gardner's syndrome


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Gardner's syndrome

 [gahrd´nerz]
familial polyposis of the colon associated with osseous and soft tissue tumors.

Gardner's syndrome

Etymology: Eldon J. Gardner, American geneticist, b. 1909
familial polyposis of the large bowel, with fibrous dysplasia of the skull, extra teeth, osteomas, fibromas, and epidermal cysts. The condition is inherited as an autosomal-dominant trait, and malignancies occur more often than usual in families having this syndrome.

Gardner's syndrome

An autosomal dominant hereditary disorder featuring multiple benign bony tumours (osteomas), EPIDERMOID CYSTS, soft tissue tumours including DESMOID TUMOURS, POLYPS in the intestine, and a 95% chance of developing cancer of the colon. (Eldon John Gardner, American geneticist, b. 1909).
References in periodicals archive ?
It is composed of colonic disorders as colonic polyps which show high potential for malignant transformation and extra colonic disorders such as osteoma, desmoid tumors, and epidermoid cysts which are benign tumors, Fortunately, osteomas and skin manifestations precede polyposis, and this facilitates early diagnosis of Gardner's syndrome and gives chance for rapid prophylactic surgical resection of colonic polyps, which is critical and lifesaving for patients with Gardner's syndrome.
Absence of colorectal polyps, skeletal abnormalities and multiple impacted or supernumerary teeth in this case, was against labelling it as Gardner's syndrome.
However it is expected of a vigilant medical professional that he actively rule out the possibility of Gardner's syndrome as a simple osteoma could be the earliest sign that heralds this syndrome which ultimately manifests as malignant polyps in gastrointestinal system.
Multiple osteomas of skull and jaw bones along with multiple epidermoid sebaceous cysts of skin, desmoids tumors, the major characteristics of gardner's syndrome were also not associated with this case.
Epidermoid inclusion cysts seen on CT of a patient with Gardner's syndrome.
1,3) The etiology is unknown, but an association with Gardner's syndrome has been described.
Gardner's syndrome: Gardner's syndrome refers to the clinical triad of adenomatous polyps of the gastrointestinal tract with a high risk of malignant transformation, epidermal cysts and dermoid tumors.
Gardner's syndrome also has an association with fibromas, lipomas, dental abnormalities, pigmented ocular fundal lesions, brain tumors and endocrine tumors.
It is one of the components of Gardner's syndrome (familial polyposis coli with desmoids tumor and craniofacial osteoma, epidermoid cyst, congenital hypertrophy of retinal pigment epithelium)