INTRAABDOMINAL DESMOID TUMORS OR FIBROMATOSIS

Intraabdominal desmoid tumour or fibromatosis, recurrent but non-metas tatic, invasive, fibroblastic proliferations, are rare tumours. From 1 968 to 1989, 16 patients were treated at Gustave Roussy Institute. The y were associated with familial adenomatous polyposis in 10 % of cases . These tumours, observed mainly in young women (70 to 85 % of cases), are aggravated by pregnancy, and spontaneous regression can occur at menopause, proving their hormonal dependance. Although histologically benign, they are serious lesions due to their invasive character ; the ir excision is complete in only 50 % of cases. They recur in 30 % to 7 5 % of cases and cause death of the patient in 30 % of cases. Treatmen t is surgical but due to their often very slow course, and their spont aneous stabilisation in some cases, a mutilating surgical treatment (e xtensive small intestine resection) does not seem to be justified. Rad iotherapy is effective only at doses incompatible with the site of the se tumours (35 to 60 Gy). Chemotherapy has never been shown to be effe ctive.