Inflammatory myofibroblastic tumors in children

Background: Inflammatory myofibroblastic tumor (IMFT) is an uncommon neoplasm in children. Methods: Retrospective review from 1993 to 2014 of patients <= 18 years of age with a histopathologic diagnosis of IMFT treated at two tertiary centers. Results: Thirty-two patientswere 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 negativemargin (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). Conclusions: IMFT 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. (C) 2016 Elsevier Inc. All rights reserved.