Desmoid tumors in familial adenomatous polyposis/Gardner's syndrome.

To clarify the clinical risk of desmoid tumors developing in familial adenomatous polyposis, we reviewed the cases of 49 Japanese patients diagnosed with familial adenomatous polyposis at our institute. In six patients who manifested desmoid tumors at a mean age of 31 years, we reviewed the clinical features and compared various phenotypic manifestations with those in the 43 patients without desmoid tumors. During the observation periods (mean, 6.5 years), two of six patients with desmoid tumors died because of the tumors, which measured > 10 cm in diameter at the initial diagnosis, whereas the remaining four patients with desmoid tumors < 5 cm did not experience complications. The patients with desmoid tumors tended to be women (5 of 6 vs. 17 of 43; p = 0.05) and more frequently had gastric fundic gland polyposis (5 of 6 vs. 17 of 43; p = 0.05) than did the patients without desmoid tumors. There were no apparent differences in other clinical manifestations, including the incidences of colonic polyposis, gastroduodenal adenomas, and extraintestinal tumors. Desmoid tumors can be serious complication in patients with familial adenomatous polyposis. There may be some association in the genesis of desmoid tumors and gastric fundic gland polyposis.

[1]  W. Bodmer,et al.  Phenotypic expression in familial adenomatous polyposis: partial prediction by mutation analysis. , 1994, Gut.

[2]  M. Mahoney,et al.  Desmoid tumors in patients with familial adenomatous polyposis , 1994, Cancer.

[3]  M. Brandi,et al.  Pharmacologic treatment of desmoid tumors in familial adenomatous polyposis: results of an in vitro study. , 1994, Surgery.

[4]  G. Petersen,et al.  Desmoid tumours in familial adenomatous polyposis. , 1994, Gut.

[5]  S. Hodgson,et al.  Genetic heterogeneity of congenital hypertrophy of the retinal pigment epithelium (CHRPE) in families with familial adenomatous polyposis. , 1994, Journal of medical genetics.

[6]  T. Iwama,et al.  Coexistence of somatic and germ-line mutations of APC gene in desmoid tumors from patients with familial adenomatous polyposis. , 1993, Cancer research.

[7]  M. Fujishima,et al.  Large intra-abdominal desmoid tumors in a patient with familial adenomatosis coli: their rapid growth detected by computerized tomography. , 1993, The American journal of gastroenterology.

[8]  N. Kemeny,et al.  Successful treatment of a desmoid tumor with doxorubicin , 1993, Cancer.

[9]  J. Church,et al.  Systemic cytotoxic chemotherapy and radiation therapy for desmoid in familial adenomatous polyposis , 1991, Diseases of the colon and rectum.

[10]  S. Altschul,et al.  Identification of FAP locus genes from chromosome 5q21. , 1991, Science.

[11]  K. Kinzler,et al.  Mutations of chromosome 5q21 genes in FAP and colorectal cancer patients. , 1991, Science.

[12]  Jagelman Dg.  Extra-colonic manifestations of familial adenomatous polyposis. , 1991 .

[13]  V. Fazio,et al.  Desmoid Tumors in Familial Polyposis Coli , 1986, Annals of surgery.

[14]  J. Reitamo,et al.  The desmoid syndrome. New aspects in the cause, pathogenesis and treatment of the desmoid tumor. , 1986, American journal of surgery.

[15]  M. Fujishima,et al.  Natural history of fundic gland polyposis in patients with familial adenomatosis coli/Gardner's syndrome. , 1985, Gastroenterology.

[16]  A. Iwashita,et al.  Fundic gland polyposis in patients without familial adenomatosis coli: its incidence and clinical features. , 1984, Gastroenterology.

[17]  W. Waddell,et al.  Nonsteroid antiinflammatory drugs and tamoxifen for desmoid tumors and carcinoma of the stomach , 1983, Journal of surgical oncology.

[18]  E. Gardner,et al.  Cancer of the lower digestive tract in one family group. , 1950, American journal of human genetics.

[19]  J. Church,et al.  Noncytotoxic drug therapy for intra-abdominal desmoid tumor in patients with familial adenomatous polyposis , 1992, Diseases of the colon and rectum.

[20]  H. Makuuchi,et al.  Intra-abdominal desmoid tumors in familial polyposis coli: A case report of tumor regression by prednisolone therapy , 1991, Diseases of the colon and rectum.