Inflammatory myofibroblastic tumors in children

BackgroundInflammatory myofibroblastic tumor (IMFT) is an uncommon neoplasm in children.MethodsRetrospective review from 1993 to 2014 of patients ≤ 18 years of age with a histopathologic diagnosis of IMFT treated at two tertiary centers.ResultsThirty-two patients were diagnosed with IMFT. Mean (± SD) age was 9.3 ± 5.7 years at diagnosis. Tumor location was variable: abdomen/pelvis (28%), head/neck region (22%), intrathoracic (22%), genitourinary (9%), bowel (6%) liver (6%), and musculoskeletal (6%). Median follow-up was 2.6 ± 4.6 years, with 3 recurrences and 2 deaths, which occurred only after recurrence. Positive microscopic margin after resection was associated with recurrence, compared to those that had a negative margin (40% vs. 0%, p = 0.04). Recurrence was associated with increased mortality (67% vs 0%, p = 0.01). Time from first symptoms to resection was shorter in those with recurrence (25.8 ± 22 vs. 179 ± 275 days, p = 0.01) and in nonsurvivors (44.0 ± 8.0 vs. 194.3 ± 53.4 days, p = 0.02). Adjuvant chemotherapy, not including steroid monotherapy, either given before or after resection, was administered more often to nonsurvivors (100% vs 4%, p = 0.009), and use of corticosteroids was also higher in the nonsurvivors (100% vs. 15%, p = 0.04).ConclusionsIMFT is a rare pediatric neoplasm with variable locations. Complete excision is critical for cure. Proposed guidelines for diagnosis, treatment and surveillance of theses tumors in children are reported.