Colorectal neoplasia in juvenile polyposis or juvenile polyps

Article Abstract:

Juvenile polyps, stemmed growths which develop in the digestive tracts of children, may occur singly or multiply (polyposis), and may be found in the stomach and small intestine as well as in the colon and rectum. Reports have varied as to whether polyposis runs in families. In contrast to polyposis, which is associated with development of tumors, single polyps are thought to be generally uncomplicated growths and so are usually treated only by surgical removal. However, several reports have cited malignant tumors in patients who had only single polyps. To better understand the implications of intestinal polyps in children, four current cases and the 57 cases of polyps or polyposis occurring between 1972 and 1980 were reviewed. Patients with inflammatory bowel disease and inflammatory polyps were excluded. All four current cases were females, aged three to seven years, and none had a family history of juvenile polyposis or colorectal cancer. Three of the four patients had adenomatous tissue (a tumorous growth) in the colorectal area. In the group of 57 patients, colorectal tumors were associated with a family history of juvenile polyps or with the occurrence of three or more polyps. These patients were most often male. One female case, like the current cases, had polyposis and a tumor but no family history, suggestive of a new mutation of the juvenile polyposis gene. It is suggested that patients with multiple juvenile polyps or a family history of the disorder should have a complete colonoscopic examination and should have periodic checkups. Their close relatives should also be examined. In addition, single juvenile polyps should also be removed, as there is a small but definite risk of tumors in these growths. (Consumer Summary produced by Reliance Medical Information, Inc.)

Prognosis, Demographic aspects, Intestinal polyps

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The molecular basis of Turcot's syndrome

Article Abstract:

Turcot's syndrome appears to result from two different genetic mutations. Turcot's syndrome is characterized by the presence of brain tumors in conjunction with multiple colon polyps. Researchers took blood samples from individuals from 14 families with at least one member who had Turcot's syndrome and tested the samples for the presence of mutations in the APC, hPMS2 and hMLH1 genes. The APC gene has traditionally been associated with familial adenomatous polyposis and the other genes are associated with hereditary nonpolyposis colon cancer. Ten of the 12 families clinically diagnosed with familial adenomatous polyposis had a mutation in the APC gene. Seventy-nine percent of the affected family members had a type of brain tumor called medulloblastoma. Up to the age of 29, these individuals had 23 times the risk of developing a brain tumor compared to the general population. Affected members in the remaining two families had mutations in the hPMS2 or hMLH1 gene. All three family members with a brain tumor had a tumor called glioblastoma multiforme.

Genetic aspects, Brain tumors

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Treatment of colonic and rectal adenomas with sulindac in familial adenomatous polyposis

Article Abstract:

The non-steroidal anti-inflammatory drug sulindac may cause intestinal polyps to regress but it may not be a substitute for surgery. Individuals with familial adenomatous polyposis develop hundreds of polyps in their colon that can ultimately turn cancerous. Many are treated surgically. Of 22 individuals with familial adenomatous polyposis, 11 took 150 milligrams of sulindac twice a day for nine months and 11 took a placebo, or inactive substance. Colonoscopies to determine the number and size of polyps were done at the beginning of the study and at three, six, nine and 12 months after. At nine months, the individuals taking sulindac had fewer than half the number of polyps as they had at the start of treatment, and the polyps were one-third the size. However, no one in the drug group experienced a complete regression of polyps. In fact, the polyps had increased slightly in number and size three months after the drug was discontinued.

Health aspects, Drug therapy, Sulindac

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