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Background: Some reports describe an increased risk for cancer in patients with the Peutz-Jeghers syndrome. Objective: To characterize occurrences of cancer in a large cohort of patients with the Peutz-Jeghers syndrome. Design: Retrospective cohort study. Setting: Tertiary care center. Patients: 34 patients with the Peutz-Jeghers syndrome identified from Mayo Clinic records from 1945 to 1996. Measurements: Cases of cancer documented by chart review and telephone follow-up. Results: 26 cases of noncutaneous cancer developed in 18 of the 34 patients: 10 cases of gastrointestinal cancer and 16 cases of extraintestinal cancer. With the use of SEER (Surveillance, Epidemiology, and End Results) data for comparison, the relative risk for cancer was 18.5 (95% CI, 8.5 to 35.2) in women with the Peutz-Jeghers syndrome and 6.2 (CI, 2.5 to 12.8) in men with the syndrome (P = 0.001). In women, the relative risk for breast and gynecologic cancer was 20.3 (CI, 7.4 to 44.2). Conclusions: The Peutz-Jeghers syndrome is associated with an increased risk for cancer. The relative risk for breast and gynecologic cancers is particularly high., Patients with Peutz-Jeghers syndrome appear to have a substantially increased risk of cancer. Peutz-Jeghers syndrome is an inherited disorder characterized by intestinal polyps and pigmented lesions on the skin and mucous membranes. Researchers reviewed the medical history of 34 patients with the syndrome. During the study period, 53% of patients developed cancer. Overall, patients had ten times the risk of cancer as people in the general population. The risk of cancer was particularly elevated for women, who had 19 times the general cancer risk.

Background: The risk for colorectal cancer among family members of patients with colorectal cancer is well established, but the risk among family members of patients with colorectal adenomas is less well established. Objective: To examine the risk for colorectal cancer among first-degree relatives of patients with adenoma compared with that among first-degree relatives of controls without adenoma. Design: Reconstructed cohort study. Setting: Three university-based colonoscopy practices in New York City. Patients: 1554 first-degree relatives of 244 patients with newly diagnosed adenomas and 2173 first-degree relatives of 362 endoscopically normal controls. Measurements: Structured interviews were used to obtain family history. Adjusted relative risks (RR) were estimated from Cox proportional hazards regression models. Results: The risk for colorectal cancer was elevated (RR, 1.74 [95% CI, 1.24 to 2.45]) among first-degree relatives of patients with newly diagnosed adenomas compared with the risk among first-degree relatives of controls. This increased risk was the same for parents (RR, 1.58 [CI, 1.07 to 2.34]) and siblings (RR, 1.58 [CI, 0.81 to 3.08]). First-degree relatives of patients with adenomas did not have elevated risk for other cancers. The risk for colorectal cancer among family members increased with decreasing age at diagnosis of adenoma in probands. Among first-degree relatives of patients who were 50 years of age or younger when the adenoma was diagnosed, the risk was more than four times greater (RR, 4.36 [CI, 2.24 to 8.51]) than that among first-degree relatives of patients who were older than 60 years of age when the adenoma was diagnosed. Conclusions: First-degree relatives of patients with newly diagnosed adenomas, particularly of patients who are 50 years of age or younger at diagnosis, are at increased risk for colorectal cancer and should undergo screening similar to that recommended for relatives of patients with colorectal cancer., Close family members of patients with colorectal adenomatous polyps may be at increased risk of colorectal cancer. Researchers interviewed 1,554 first-degree relatives of 244 patients with the benign colon tumors and 2,173 relatives of 362 people without growths in the colon. The risk of ultimately developing colorectal cancer was 74% greater in the parents and siblings of those with colorectal polyps. People with a close family history of polyposis or colon cancer should be screened regularly for growths in the colon.

Bone morphogenetic protein receptor 1A is the second gene to be linked to juvenile polyposis, a condition in which patients develop gastrointestinal polyps that increase their risk of GI cancer [...]

Familial adenomatous polyposis (FAP) is an inherited condition of multiple polyps in the colon and rectum that is associated with a high risk for malignancy. This condition is often treated with excision of a portion of the colon (colectomy). Vitamin C has been shown to inhibit the growth of colon-rectal tumors. It has been reported that following surgery, some FAP patients given vitamin C had reduced rectal polyp density. The inconsistent effect of vitamin C observed in these patients may be due to the use of slow-release forms of the vitamin and its rapid transit through the intestine, which reduces contact of vitamin C with the mucosa. Additionally, patients may alter their diet following surgery, producing a subclinical vitamin C deficiency. A study was conducted of 55 healthy, post-colectomy FAP patients. Levels of ascorbic acid (vitamin C) in leukocytes (white blood cells) were measured in the FAP patients, in 17 patients with chronic disease, and in 28 normal control patients. Patients taking vitamin C supplements were excluded from the study. Results showed low leucocyte vitamin C levels in FAP patients. This low level may represent a defect in vitamin C absorption. It is suggested that the use of slow-release vitamin C is less effective than other vitamin C preparations. (Consumer Summary produced by Reliance Medical Information, Inc.)

Colorectal cancer seldom develops before age 20 in patients with familial adenomatous polyposis, and the cases that occur before that age are associated with severe, symptomatic colonic disease, said Dr. [...]