title: Analysis of clinical features and outcomes for inflammatory myofibroblastic tumors in China: 11 years of experience at a single center.
abstract: OBJECTIVE
  Inflammatory myofibroblastic tumor (IMT) is a rare benign neoplasm. The purpose of this study was to review the clinical characteristics, imaging and pathological features, and outcomes of children with IMTs from a single center in China.
  
  
  METHODS
  A retrospective file review was conducted involving 23 cases of pathologically confirmed IMTs treated at the Children's Hospital between April 2003 and April 2014.
  
  
  RESULTS
  The tumor locations included multiple anatomic sites, as follows: abdomen or pelvis (n = 17); lungs (n = 2); head and neck (n = 1); trunk (n = 1); and extremities (n = 2). The tumors were associated with various clinical presentations. The predominant symptoms included an anemic appearance, fevers, and an asymptomatic mass. Computed tomography scanning showed solid, heterogeneous, well-demarcated masses; the appearance of enhancement was variable. MRI appeared hypointense on T1-weighted images and hypointense or hyperintense on T2-weighted images. Immunohistochemical staining revealed anaplastic lymphoma kinase was negative in 11 of 13 cases tested. One patient quit treatment for the unresectable mass after biopsy and died 2 years later, and another patient with incompletely resection is alive at 30 months following chemotherapy. The remaining 21 cases had complete resections; one patient died due to a recurrence, and the other 20 patients survived and were tumor free. The follow-up ranged from 7 to 141 months, with a mean of 56 months. The 3-year OS was 88 % (95 % CI, 57-97 %).
  
  
  CONCLUSIONS
  IMT is a benign neoplasm that rarely presents with malignant features. Complete resection is curative in most patients. ALK+ is variable for diagnosis. Close follow-up is necessary for patients who undergo surgical resection.
authors.affiliation: Department of Pediatric Surgery, Children's Hospital of Fudan University, 399 Wan yuan Road, Shanghai, 201102, People's Republic of China.
authors.forename: Zuopeng
authors.initials: Z
authors.lastname: Wang
authors.affiliation: Department of Endocrinology, Huashan Hospital of Fudan University, Shanghai, People's Republic of China.
authors.forename: Xiaolong
authors.initials: X
authors.lastname: Zhao
authors.affiliation: Department of Pediatric Surgery, Children's Hospital of Fudan University, 399 Wan yuan Road, Shanghai, 201102, People's Republic of China. likai2727@163.com.
authors.forename: Kai
authors.initials: K
authors.lastname: Li
authors.affiliation: Department of Pediatric Surgery, Children's Hospital of Fudan University, 399 Wan yuan Road, Shanghai, 201102, People's Republic of China.
authors.forename: Wei
authors.initials: W
authors.lastname: Yao
authors.affiliation: Department of Pediatric Surgery, Children's Hospital of Fudan University, 399 Wan yuan Road, Shanghai, 201102, People's Republic of China.
authors.forename: Kuiran
authors.initials: K
authors.lastname: Dong
authors.affiliation: Department of Pediatric Surgery, Children's Hospital of Fudan University, 399 Wan yuan Road, Shanghai, 201102, People's Republic of China.
authors.forename: Xianmin
authors.initials: X
authors.lastname: Xiao
authors.affiliation: Department of Pediatric Surgery, Children's Hospital of Fudan University, 399 Wan yuan Road, Shanghai, 201102, People's Republic of China.
authors.forename: Shan
authors.initials: S
authors.lastname: Zheng
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D002681:China; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D008279:Magnetic Resonance Imaging; D008297:Male; D047708:Myofibroma / Q000000981:diagnostic imaging* / Q000473:pathology* / Q000628:therapy; D017063:Outcome Assessment, Health Care / Q000706:statistics & numerical data*; D012189:Retrospective Studies; D014057:Tomography, X-Ray Computed
keywords: ALK; Follow-up; Inflammatory myofibroblastic tumor; Survival analysis; Treatment