Problems in diagnosis and management of desmoid tumors.

Thirty-two primary desmoid tumors occurred in 29 patients. The median patient age was 32 years, and 55% of the patients were females. An antecedent history of trauma at the tumor site was elicited from 28% of patients. Thirty-one of 32 primary tumors were completely excised at initial presentation. Five tumors were treated with adjuvant radiotherapy. The overall recurrence rate for primary and recurrent lesions was 60%. The recurrence rate in children (88%) was more than twice that found in adults (38%). A single recurrence did not significantly increase the likelihood of a subsequent recurrence. Greater than 90% of all recurrences took place within 3 years of treatment. The rate of recurrence was not clearly influenced by the status of histologic margins, although this was examined in less than half the tumors. Desmoid tumors are aggressive neoplasms that exhibit a strong propensity for local recurrence. They should be treated as low-grade malignancies with documentation of histologic margins and close clinical follow-up within the framework of a tumor registry.