title: Myofibroma in Infancy and Childhood.
abstract: Myofibromas are rare fibroblastic-myofibroblastic tumors in children. Their biological behavior is unpredictable and spontaneous regressions have been described. This is a retrospective review of clinical characteristics, treatment, and outcome of children diagnosed with myofibroma between 1999 and 2013 at Texas Children's Hospital. The median age at diagnosis of 42 patients was 37 months. Approximately two thirds of the patients were male. The median length of follow-up was 50.5 months (range, 0 to 165 mo). Thirty-eight patients (90%) had solitary lesions; 19 (50%) in the head and neck, 10 (26%) in the limbs, and 9 (24%) in the trunk. Twelve patients underwent a complete surgical resection. Of the 30 patients with positive margins, only 1 had tumor progression. Two patients had multicentric involvement, and 2 patients had generalized disease with visceral involvement. One patient with generalized disease and a progressive maxillary sinus mass was treated with vinblastine and methotrexate chemotherapy followed by complete surgical resection. All patients were alive at last follow-up. Myofibromas of childhood demonstrate clinical variability, and may spontaneously regress. Positive surgical margins do not adversely affect outcome. The rare patient with progressive unresectable disease may benefit from chemotherapy.
authors.affiliation: *Department of Pediatrics, Baylor College of Medicine, Texas Children's Cancer Center, Texas Children's Hospital †Department of Pathology and Immunology, Baylor College of Medicine, Houston, TX.
authors.forename: Priya
authors.initials: P
authors.lastname: Mahajan
authors.forename: John
authors.initials: J
authors.lastname: Hicks
authors.forename: Murali
authors.initials: M
authors.lastname: Chintagumpala
authors.forename: Rajkumar
authors.initials: R
authors.lastname: Venkatramani
mesh_terms: D000293:Adolescent; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D047708:Myofibroma / Q000473:pathology* / Q000601:surgery / Q000628:therapy*; D012189:Retrospective Studies; D013514:Surgical Procedures, Operative; D013781:Texas; D016896:Treatment Outcome