Title

Inflammatory myofibroblastic tumors in children.

Document Type

Article

Publication Date

4-1-2016

Identifier

DOI: 10.1016/j.jpedsurg.2015.11.015

Abstract

BACKGROUND: Inflammatory myofibroblastic tumor (IMFT) is an uncommon neoplasm in children.

METHODS: Retrospective review from 1993 to 2014 of patients ≤18years of age with a histopathologic diagnosis of IMFT treated at two tertiary centers.

RESULTS: Thirty-two patients were diagnosed with IMFT. Mean (±SD) age was 9.3±5.7years 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±4.6years, 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±275days, p=0.01) and in nonsurvivors (44.0±8.0 vs. 194.3±53.4days, 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.

Journal Title

Journal of pediatric surgery

Volume

51

Issue

4

First Page

541

Last Page

544

MeSH Keywords

Adolescent; Antineoplastic Agents; Chemotherapy, Adjuvant; Child; Child, Preschool; Female; Follow-Up Studies; Granuloma, Plasma Cell; Humans; Infant; Infant, Newborn; Male; Retrospective Studies; Treatment Outcome

Keywords

Cancer; Children; Teenagers; Tumors

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