APSA Paper
Desmoid fibromatosis in children and adolescents: A conservative approach to management

https://doi.org/10.1016/j.jpedsurg.2012.10.017Get rights and content

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.

Section snippets

Methods

With Institutional Review Board approval, we performed a retrospective review of our institutional pathology database to identify all patients under 21 years of age with desmoid fibromatosis treated between 1970 and 2010. A total of 96 patients with desmoid tumors were identified. Three patients were excluded from the study for incomplete follow-up; the remaining 93 patients were included in the final analysis. The electronic medical records of all patients were reviewed for data on

Patient demographics, tumor characteristics, and treatment

A total of 93 patients with desmoid fibromatosis were treated during the study period, with an age at diagnosis ranging from 2 months to 21 years (median, 16.1 years). Median follow-up for the group was 6.4 years (range, 11 months–25 years). The male-to-female ratio was 1.1:1. Tumor site distribution was as follows: 60 (65%) in the extremities, 15 (16%) intra-abdominal, 13 (14%) in the thoracic or abdominal wall, and 5 (5%) in the head and neck. Ten patients (11%) had a past medical history of

Discussion

The management of desmoid tumor is complex. While some tumors are effectively managed with low-risk surgical resection, patients with the most rapidly growing or symptomatic tumors may receive multimodal treatment that incorporates aggressive surgical resection, adjuvant systemic chemotherapy, and/or radiation. Unfortunately, aggressive surgical therapy can be complicated by significant morbidity. Our results support previous findings that surgery provides the best opportunity for long-term

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