Desmoid tumors and mesenteric fibromatosis in Gardner's syndrome: report of kindred 109.
Kindred 109, from which Gardner's syndrome was first described, now has 224 members, 28 of whom have inherited the syndrome. Among the group of 28, desmoid tumors or mesenteric fibromatosis or both have developed in eight (29%). In four patients, the fibrous dysplasia appeared to originate in postsurgical abdominal incisional scars; in two of these cases, the mesenteric involvement resulted in death. In one patient, the process was present in the mesentery at the original operation. In three patients, the fibrous dysplastic lesions were extraincisional, with no known trauma associated with their occurrence.
D000009:Abdominal Muscles / Q000473:pathology; D000328:Adult; D005260:Female; D005350:Fibroma / Q000235:genetics* / Q000473:pathology; D005500:Follow-Up Studies; D006801:Humans; D007417:Intestinal Polyps / Q000235:genetics* / Q000473:pathology; D007422:Intestines / Q000473:pathology; D008297:Male; D008643:Mesentery / Q000473:pathology; D010534:Peritoneal Neoplasms / Q000235:genetics* / Q000473:pathology; D013577:Syndrome