BACKGROUND: This study examines the natural history of patients treated for multifocal desmoids at a referral center for sarcomas. METHODS: Patients with multifocal desmoids seen at Memorial Sloan-Kettering Cancer Center in the 9-year period between 1982 and 1991 were identified through a prospective sarcoma data base. The clinical histories were reviewed. In addition, tumor estrogen and progesterone receptor status were evaluated by immunohistochemistry. RESULTS: This rare condition occurred in six (4.8%) of the 124 patients with desmoid tumors and all occurred in premenopausal women. In each instance the tumors were restricted to one anatomic region of the body and no patient had familial polyposis or Gardner's syndrome. When tested immunohistochemically, the tumors displayed no nuclear reactivity for estrogen or progesterone receptors. CONCLUSIONS: Further investigative work must be undertaken before considering routine use of antiestrogen therapy even in this subset of patients with desmoid tumors.