title: Outcomes of pulmonary metastases in hepatoblastoma--is the prognosis always poor?
abstract: BACKGROUND
  Hepatoblastoma is a rare tumour accounting for approximately 1% of all paediatric malignancies. Hepatoblastoma complicated by pulmonary metastatic disease continues to cause management difficulties due to a lack of robust evidence and treatment guidelines.
  
  
  METHODS
  This series is the experience of a tertiary paediatric referral centre. Patients were prospectively enlisted, and their charts were retrospectively reviewed.
  
  
  RESULTS
  Thirty-seven patients were treated for hepatoblastoma from 1995 to 2012 inclusive. The overall survival was 34/37(91.9%). Eight patients had lung metastases at diagnosis (LMD) and twenty-nine did not (NLMD). Two-year EFS was 62.5% in the LMD group and 89.3% in the NLMD group (p=0.078). Overall survival in the LMD and NLMD groups was 100% and 89.7%, respectively (p=0.389). Two patients in the LMD group required multiple thoracic resections to achieve cure. Within the NLMD group, two patients developed lung metastases whilst on treatment, and both of these patients died.
  
  
  CONCLUSIONS
  In this series, children presenting with lung metastases had a higher risk of relapse but excellent overall survival. However, children who developed pulmonary disease during treatment had a poor prognosis. We advocate aggressive surgical treatment of pulmonary hepatoblastoma to achieve cure.
authors: Douglas Cohen Department of Paediatric Surgery, The Children's Hospital at Westmead, NSW 2145, Australia.
authors: Dylan
authors: D
authors: Wanaguru
authors: Albert
authors: A
authors: Shun
authors: Neil
authors: N
authors: Price
authors: Jonathan
authors: J
authors: Karpelowsky
mesh_terms: D000970:Antineoplastic Agents / Q000008:administration & dosage; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D017024:Chemotherapy, Adjuvant; D002648:Child; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D006498:Hepatectomy; D018197:Hepatoblastoma / Q000175:diagnosis / Q000401:mortality / Q000556:secondary* / Q000628:therapy; D006801:Humans; D007223:Infant; D008113:Liver Neoplasms / Q000175:diagnosis / Q000401:mortality / Q000473:pathology* / Q000628:therapy; D008175:Lung Neoplasms / Q000175:diagnosis / Q000401:mortality / Q000556:secondary* / Q000628:therapy; D008297:Male; D020360:Neoadjuvant Therapy; D011379:Prognosis; D012189:Retrospective Studies; D016019:Survival Analysis; D013908:Thoracotomy; D016896:Treatment Outcome
chemical_list: D000970:Antineoplastic Agents
keywords: Hepatoblastoma; Metastases; Pulmonary