title: Inflammatory myofibroblastic tumors in children.
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.
authors.affiliation: Children's Mercy Hospital, Kansas City, MO, United States.
authors.forename: Brian G A
authors.initials: BG
authors.lastname: Dalton
authors.affiliation: Children's Mercy Hospital, Kansas City, MO, United States.
authors.forename: Priscilla G
authors.initials: PG
authors.lastname: Thomas
authors.affiliation: Children's Mercy Hospital, Kansas City, MO, United States.
authors.forename: Nicole E
authors.initials: NE
authors.lastname: Sharp
authors.affiliation: Children's Mercy Hospital, Kansas City, MO, United States.
authors.forename: Michelle A
authors.initials: MA
authors.lastname: Manalang
authors.affiliation: Mayo Clinic Division of Pediatric Surgery, Rochester, MN, United States.
authors.forename: James E
authors.initials: JE
authors.lastname: Fisher
authors.affiliation: Mayo Clinic Division of Pediatric Surgery, Rochester, MN, United States.
authors.forename: Christopher R
authors.initials: CR
authors.lastname: Moir
authors.affiliation: Children's Mercy Hospital, Kansas City, MO, United States.
authors.forename: Shawn D
authors.initials: SD
authors.lastname: St Peter
authors.affiliation: Children's Mercy Hospital, Kansas City, MO, United States. Electronic address: ciqbal@cmh.edu.
authors.forename: Corey W
authors.initials: CW
authors.lastname: Iqbal
mesh_terms: D000293:Adolescent; D000970:Antineoplastic Agents / Q000627:therapeutic use; D017024:Chemotherapy, Adjuvant; D002648:Child; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D006104:Granuloma, Plasma Cell / Q000175:diagnosis / Q000188:drug therapy / Q000401:mortality / Q000601:surgery; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D012189:Retrospective Studies; D016896:Treatment Outcome
chemical_list: D000970:Antineoplastic Agents
keywords: Children; Inflammatory myofibroblastic tumor; Inflammatory pseudotumor; Negative surgical margin; Pediatric; Recurrence