Gardners Syndrome

Gardner's syndrome was originally described in individuals with FAP-like polyps who had additional findings outside the GIT, including epidermal cysts and subcutaneous fibromas of the skin, desmoids of the skin and abdomen, osteomas, dental abnormalities, pigmented patches in the retina and tumours of other organs [4]. Gardner's syndrome includes a variety of manifestations: Soft-tissue tumours:

• Epidermoid inclusion cysts of the skin

• Dermoid tumours

• Lipofibromas

• Neurofibromas

• Leiomyomas

• Mammary fibromatosis

• Intra-abdominal desmoid tumours and peritoneal fibromas

GI tumours:

• Colic polyposis (nearly 100% precancerosis)

• Gastric adenomatous polyps

• Gastric hamartomatous polyps

• Duodenal adenomatous polyps

• Periampullary carcinoma

• Hepatoblastoma

• Pancreatic carcinoma

• Lymphoid hyperplasia of the terminal ileum Osseous abnormalities:

• Usually involving the membranous bones, mandible, calvaria, maxilla, ribs and long bones

• Self-limited benign exostosis

• Bone islands and periosteal thickening Endocrine tumours:

• Thyroid carcinoma (papillary)

• Carcinoid tumours of the small bowel

• Parathyroid adenoma

• Adrenal adenoma/carcinoma

• Pituitary chromophobe adenoma Central nervous system medulloblastoma; Abnormal dentition:

• Supernumerary teeth

• Impacted teeth

• Hypercementosis

• Teeth more prone to carries

The distinction between Gardner's syndrome and FAP was initially unclear, however, because some individuals diagnosed with FAP also had extraintestinal signs. Clinically, Gardner's syndrome and FAP can occur in the same kindred. It was subsequently found that both conditions can result from the same mutation in the APC gene. Thus, Gardner's syndrome and FAP are allelic. [Many genes show alterations in GIT neoplasia that do not, as yet, have an immunohistochemically detectable protein product. These genes include the APC (adenomatous polypo-sis coli) and the MCC (mutated in colon cancer) genes].

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