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