Desmoid fibromatosis in children and adolescents: a conservative approach to management

Abstract

Purpose: Desmoid fibromatosis is associated with frequent recurrence and significant morbidity, but no metastases. To examine the impact of initial non-operative management on event-free survival (EFS) in children, we reviewed our institutional experience with this tumor.

Methods: We retrospectively reviewed our institutional database for pediatric cases of desmoid fibromatosis treated between 1970 and 2010. Survival was analyzed using the Kaplan-Meier method and log-rank test.

Results: Ninety-three patients were identified, with a median follow-up of 6 years. Median age at diagnosis was 16 years. Forty-seven patients presented with primary tumors, and forty-six had recurrent or progressing disease. Five-year OS was 100%, and 5-year EFS was 31.8%, with a median time to event of 1.48 years. There was no significant difference in 5-year EFS between patients who were managed expectantly and those who initially received treatment (21% versus 34%, P=.09). Sex, race, history of trauma, or familial adenomatous polyposis, multifocality, tumor size, tumor location, and resection status did not correlate with EFS.

Conclusion: Our findings support a conservative initial approach in the management of desmoid fibromatosis. In patients at risk for morbid procedures, upfront resection should be reserved for select tumors that demonstrate aggressive growth or cause serious symptoms.