title: Hepatoblastoma--evolution of management and outcome and significance of histology of the resected tumor. A 31-year experience with 40 cases.
abstract: OBJECTIVE
  The aim of this study was to retrospectively evaluate and compare the clinical features, treatment strategy, pathology, and outcome of all patients with hepatoblastoma treated at an African hospital over a 31-year period (1970 to 2001).
  
  
  METHODS
  Forty patients with hepatoblastoma were divided into 3 groups according to the treatment given. Group I (1970 to 1983, 14 patients) had no protocol therapy; group II (1984 to 1988, 6 patients) received protocol treatment according to Children's Study Group (CCSG) guidelines; group III (1989 to 2001, 20 patients) received SIOPEL protocol therapy. All available clinical, surgical, radiologic, and pathologic data were reviewed and analyzed.
  
  
  RESULTS
  Overall patient survival was as follows: group I, 14%; group II, 50%, and group III, 80%. Deaths in group II were caused by chemotherapy-induced immunosuppression only. Prognostic data for group III showed that all tumor-related deaths could be predicted by identifying multifocal disseminated growth patterns (P =.001) or vascular invasion (P =.001) in resected tumors. Of the 40 diagnostic tumor biopsies performed, 2 significant complications (1 death, 1 intraperitoneal tumor seeding) occurred. Histologic criteria evaluating these biopsies were not predictive of overall survival.
  
  
  CONCLUSIONS
  The introduction of protocol therapy has resulted in a marked improvement in survival. Immunosuppression-related sepsis in our setting resulted in unacceptable mortality in patients treated according to CCSG guidelines. A diagnostic biopsy in hepatoblastoma is of value but not without complications. Preoperative chemotherapy followed by complete surgical excision according to International Society of Paediatric Oncology guidelines yields excellent results with a current survival rate of 80%.
authors.affiliation: Department of Surgery, Red Cross Children's Hospital, Cape Town, South Africa.
authors.forename: J Q
authors.initials: JQ
authors.lastname: Davies
authors.forename: P M
authors.initials: PM
authors.lastname: de la Hall
authors.forename: R O C
authors.initials: RO
authors.lastname: Kaschula
authors.forename: C C
authors.initials: CC
authors.lastname: Sinclair-Smith
authors.forename: P
authors.initials: P
authors.lastname: Hartley
authors.forename: H
authors.initials: H
authors.lastname: Rode
authors.forename: A J W
authors.initials: AJ
authors.lastname: Millar
mesh_terms: D000971:Antineoplastic Combined Chemotherapy Protocols / Q000008:administration & dosage / Q000009:adverse effects / Q000627:therapeutic use*; D019090:Case Management / Q000639:trends; D002675:Child, Preschool; D002945:Cisplatin / Q000008:administration & dosage; D003131:Combined Modality Therapy; D004317:Doxorubicin / Q000008:administration & dosage; D005260:Female; D006498:Hepatectomy; D018197:Hepatoblastoma / Q000000981:diagnostic imaging / Q000188:drug therapy / Q000401:mortality / Q000556:secondary / Q000601:surgery*; D006801:Humans; D016867:Immunocompromised Host; D007223:Infant; D007231:Infant, Newborn; D008113:Liver Neoplasms / Q000000981:diagnostic imaging / Q000188:drug therapy / Q000401:mortality / Q000473:pathology / Q000601:surgery*; D008175:Lung Neoplasms / Q000556:secondary; D008297:Male; D020360:Neoadjuvant Therapy; D009367:Neoplasm Staging; D011379:Prognosis; D011859:Radiography; D012189:Retrospective Studies; D018805:Sepsis / Q000209:etiology / Q000401:mortality; D013019:South Africa / Q000453:epidemiology; D016019:Survival Analysis; D016896:Treatment Outcome; D047368:Tumor Burden
chemical_list: D004317:Doxorubicin; D002945:Cisplatin