title: [Comprehensive treatment of neuroblastoma in children associated with opsoclonus-myoclonus-ataxia syndrome].
abstract: OBJECTIVE
  To investigate the efficacy of combined modality therapy for neuroblastoma in children associated with opsoclonus-myoclonus syndrome (OMS-NB).
  
  
  METHODS
  From May 2011 to December 2013, 6 consecutive patients (4 boys and 2 girls) diagnosed as OMS-NB underwent surgery and chemotherapy in the First Hospital, Peking University. The median age of onset was 19.5 months (range 13-24 months) and misdiagnosis occurred 7.5 months (range 2-14 months) ago. A retrospective analysis for the location, stage, pathological type, treatment way and outcome of neuroblastoma was done.
  
  
  RESULTS
  (1) All patients were misdiagnosed as simply opsoclonus-myoclonus syndrome (OMS) at the time of onset. They had been receiving treatment with adrenocorticotropic hormone and intravenous immunoglobulin within 1-13 months.OMS-NB was diagnosed by means of enhanced abdominal CT image which was delayed to be given after the poor efficacy or relapse. (2) The primary tumors were almost all small, stage I-II, located in adrenal, retroperitoneal or pelvis. The pathology of tumors included ganglioneuroblastoma (5/6) and neuroblastoma (1/6). (3) All these cases underwent surgery, 4/6 cases with complete tumor resection, 2/6 cases with tumor around the aorta and induced local residue. Preoperative and postoperative chemotherapy was given to 2 and 5 cases, respectively. (4) The patients were followed up for 3-31 months, except 1 patient lost, the other 5 are currently surviving disease-free (3 having been at the end of chemotherapy, 1 still in chemotherapy, and another had local recurrence and is receiving radiotherapy and chemotherapy after the second operation and now also stopped taking the medicine). The symptoms of nervous system have been significantly improved during postoperative chemotherapy.
  
  
  CONCLUSIONS
  To reduce the misdiagnosis, regular CT imaging of the abdomen or pelvic should be ordered for all cases with OMS. The children with OMS-NB need to be actively treated with the combined modality therapy including surgery, chemotherapy or radiotherapy, to reduce recurrence and reduce the symptoms of nervous system.
authors.affiliation: Department of Pediatrics, First Hospital, Peking University, Beijing 100034, China.
authors.forename: Weihong
authors.initials: W
authors.lastname: Zhao
authors.forename: Qing
authors.initials: Q
authors.lastname: Sun
authors.forename: Yao
authors.initials: Y
authors.lastname: Xie
authors.affiliation: Email: huayingcxq@hotmail.com.
authors.forename: Ying
authors.initials: Y
authors.lastname: Hua
authors.forename: Hui
authors.initials: H
authors.lastname: Xiong
authors.forename: Jun
authors.initials: J
authors.lastname: Jia
authors.forename: Xintian
authors.initials: X
authors.lastname: Lu
mesh_terms: D000310:Adrenal Gland Neoplasms / Q000150:complications / Q000175:diagnosis / Q000601:surgery / Q000628:therapy*; D000970:Antineoplastic Agents / Q000008:administration & dosage; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D015415:Biomarkers / Q000032:analysis; D017024:Chemotherapy, Adjuvant; D002675:Child, Preschool; D003131:Combined Modality Therapy; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D008297:Male; D009364:Neoplasm Recurrence, Local / Q000601:surgery / Q000628:therapy; D009367:Neoplasm Staging; D009447:Neuroblastoma / Q000150:complications / Q000175:diagnosis / Q000601:surgery / Q000628:therapy*; D053578:Opsoclonus-Myoclonus Syndrome / Q000175:diagnosis / Q000209:etiology / Q000628:therapy*; D012186:Retroperitoneal Neoplasms / Q000150:complications / Q000175:diagnosis / Q000601:surgery / Q000628:therapy; D012189:Retrospective Studies; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome
chemical_list: D000970:Antineoplastic Agents; D015415:Biomarkers