title: [Langerhans cell histiocytosis of the temporal bone in children: 7 cases analysis].
abstract: OBJECTIVE
  To analyze the clinical manifestations, diagnosis, therapy and prognosis of Langerhans cell histiocytosis (LCH) of the temporal bone in children.
  
  
  METHODS
  Seven children with LCH of the temporal bone n our hospital were retrospectively summed up from April 2009 to April 2014. The patients were followed up 1-5 years, their clinical manifestations, imaging findings, diagnosis, therapy and prognosis were studied. Correlation between clinical classifications and prognosis was also analyzed.
  
  
  RESULTS
  Among the 7 patients, 4 were boys and 3 were girls. 5 cases belonged to the single system group and 2 cases belonged to the multisystem group. The most common clinical characters were temporal tumor, otorrhea, otalgia, hearing loss and granulation of external auditory canal. CT of the temporal bones showed extensive osteolytic destructions with diffuse soft tissure density, without border sclerotization. The cases were. received different therapies. Followed up for 1-5 years, 4 cases were regressive, 1 case kept stable, while 2 cases showed progressive. The two boys then received standard treatment combined steroids with vinblastine. The prognosis in the multisystem group was significantly different from the single system group (P < 0.05).
  
  
  CONCLUSIONS
  The clinical manifestations of LCH vary a lot. The diagnosis is based on histological and immunophenotypic examination of lesion tissue. The main therapy includs surgery, chemotherapy and radiotherapy. The prognosis of the single system group is much better than the multisystem group.
authors.forename: Xiaoli
authors.initials: X
authors.lastname: Wang
authors.forename: Wei
authors.initials: W
authors.lastname: Liu
authors.forename: Shumin
authors.initials: S
authors.lastname: Xie
authors.forename: Anquan
authors.initials: A
authors.lastname: Peng
authors.forename: Jihao
authors.initials: J
authors.lastname: Ren
mesh_terms: D002648:Child; D003131:Combined Modality Therapy; D003638:Deafness / Q000209:etiology; D004424:Ear Canal / Q000473:pathology; D004427:Ear Diseases / Q000209:etiology; D005260:Female; D006646:Histiocytosis, Langerhans-Cell / Q000175:diagnosis* / Q000473:pathology* / Q000628:therapy*; D006801:Humans; D008297:Male; D011379:Prognosis; D012189:Retrospective Studies; D013256:Steroids / Q000627:therapeutic use; D013701:Temporal Bone / Q000473:pathology*
chemical_list: D013256:Steroids