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Gardner’s syndrome is an autosomal dominant syndrome of adenomatous polyposis of the colon that is associated with osteomas and skin lesions. The osteomas often precede any other symptoms, including those due to colonic polyposis, and may thus serve as a marker of the latter.
The association of colonic polyps with colonic cancer in almost 100% of patients with Gardner’s syndrome makes it important to screen patients with osteomas for the syndrome, and thus potentially save their lives. Asking for a family history is the first step; however, even in its absence, a detailed assessment is highly recommended in trying to establish this condition in an asymptomatic patient. This is especially important since about a quarter of these patients have no family history of the disease—the mutation apparently having arisen de novo.
The consideration of this diagnosis means that the gastric mucosa, the thyroid, retinal epithelium, skull, and teeth, as well as the skin, need to be assessed for epidermoid cysts, desmoid tumors, congenital hypertrophy of the retinal pigment epithelium, odontomes, impacted teeth, and colonic polyps. The classical site for osteoma formation is the mandible, especially at the angle of the jaw, but these tumors may also form on the skull, the paranasal sinuses, and long bones. A CT scan may help show their location and size.
Polyps are most often found to blanket the entire colonic surface but may sometimes occur in the gastric and small intestinal mucosa. They are usually first seen after puberty but become symptomatic at about age 40, on average. Colonic adenocarcinoma is the inevitable outcome by this age in all patients with Gardner syndrome.
Screening for papillary carcinoma of the thyroid, adenomas, and adenocarcinomas of the adrenal glands, hepatocellular carcinomas, osteosarcoma, and chondrosarcoma, and for other thyroid and liver tumors is also an important part of the initial evaluation.
Because all patients develop colonic cancer before 40 years of age, a prophylactic colectomy is necessary. Various surgical options for preventing colon cancer are available, incuding:
A restorative proctocolectomy with ileal pouch anal anastomosis (RPC/IPAA) along with mucosectomy is recommended because the whole of the mucosa of the large intestine is resected to prevent polyp formation while still conserving intestinal function and enabling sexual fulfilment. By avoiding the need for a colostomy, the patient is able to obviate a lot of psychological and physical distress. An ileostomy is put in place temporarily to allow the rectal-colonic anastomosis to heal properly.
When gastric polyps are found in association with Gardner’s syndrome, the rate and speed of carcinogenesis in such cases is lower than for colorectal polyposis. As such, a more conservative approach, such as snare polypectomy, may yield good results.
Cutaneous cysts of various types are treated as they would be in normal patients. Osteomas which cause disfigurement or functional interference may be resected. Desmoid tumors recur in eight of ten cases following resection, and hence this should be performed only if the patient has symptoms. Various drugs have been tried, from the NSAID category, selective estrogen receptor modulators, and even cytotoxic drugs, in situations where the tumor was spreading fast or could not be resected. Radiation therapy has also been attempted. Mesenteric desmoids are extremely difficult to manage by surgery because of the diffuse nature of the growth and its interference with neighboring vessels and organs.
Drugs such as non-steroidal anti-inflammatory drugs (NSAIDs) may be of help in preventing the progression of colorectal polyps to carcinoma in familial adenomatous polyposis of which Gardner’s syndrome is one form. They have been shown to reduce the size and number of polyps for the duration of administration, but this is not invariable, with some patients showing the emergence of new tumors or the malignant transformation of existing polyps at the same time.
Newer drugs in this category include the COX-2 inhibitors because of the finding that COX-2 receptors are present in colonic mucosa undergoing cancerous changes but not in the normal colonic mucosa. The result of clinical trials showed that polyp size was decreased, but the effect on malignancy rates has not been determined.
Some claims have been made for the use of traditional Chinese medicine with surgical polypectomy of gastric polyps, suggesting a beneficial effect in terms of their disappearance over a short period of time. The characterization and testing of the components of traditional Chinese medicine remains a vital but missing link in making use of this suggestion in treatment recommendations. It is known that these contain an array of organic acids such as oxalic, tartaric, malic, and lactic acid, along with palmitic acid, uracil, and erythritol derivatives.
Other herbal alternatives such as green tea extract and ascorbic acid have not been proven to be effective in this regard.
All patients with Gardner’s syndrome must receive scheduled regular monitoring of the small intestine and colon as well as the liver and thyroid gland. It is indicated when:
Thyroid examination and ultrasound must be carried out yearly, and liver monitoring using ultrasound and AFP (alfa-fetoprotein) measurements.