title: Surgical view of the treatment of patients with hepatoblastoma: results from the first prospective trial of the International Society of Pediatric Oncology Liver Tumor Study Group.
abstract: BACKGROUND
  Surgical resection is the cornerstone of treatment for patients with hepatoblastoma (HB). The Society of Pediatric Oncology Liver Tumor Study Group launched its first prospective trial (SIOPEL-1) with the intention to treat all patients with preoperative chemotherapy and delayed surgical resection. The objective of this article was to assess the assumed surgical advantages of primary chemotherapy.
  
  
  METHODS
  Between 1990 and 1994, 154 patients age < 16 years with HB were registered on SIOPEL-1. The pretreatment extent of disease was assessed, and, after undergoing biopsy, patients were treated with cisplatin 80 mg/m(2) intravenously over 24 hours and doxorubicin 60 mg/m(2) intravenously over 48 hours by continuous infusion (PLADO). Generally, tumors were resected after four of a total of six courses of PLADO.
  
  
  RESULTS
  One hundred twenty eight patients underwent surgical resection (13 patients underwent primary surgery, and 115 patients underwent delayed surgery after PLADO). A pretreatment surgical biopsy was performed in 96 of 128 patients (75%). Biopsy complications occurred in 7 of 96 patients (7%). Twenty-two patients showed pulmonary metastases at the time of diagnosis, and 7 patients underwent thoracotomy. Operative morbidity and mortality were 18% and 5%, respectively. Complete macroscopic surgical resection was achieved in 106 patients (92%), including 6 patients who underwent orthotopic liver transplantation. The actuarial 5-year event free survival (EFS) rate for all 154 patients in the study was 66%, and the overall survival (OS) rate was 75%. For the 115 patients who were included in the surgical analysis that followed the exact protocol, the EFS and OS rates were 75% and 85%, respectively.
  
  
  CONCLUSIONS
  Biopsy is a safe procedure and should be performed routinely. Preoperative chemotherapy seems to make tumor resection easier. Reresection of a positive resection margin does not necessarily have to be performed, because postoperative chemotherapy showed good results. Resection of lung metastases can be curative if there is local control of the primary tumor; however, results showed that the patient's prognosis was worse. Surgical morbidity or mortality rates were not necessarily higher in large multicenter studies. More importantly, countries of lesser economic status also can contribute effectively to these trials.
authors.affiliation: Pediatric Surgical Center Amsterdam (EKZ-AMC/VUmc), Amsterdam, The Netherlands.
authors.forename: J Marco
authors.initials: JM
authors.lastname: Schnater
authors.forename: Daniël C
authors.initials: DC
authors.lastname: Aronson
authors.forename: Jack
authors.initials: J
authors.lastname: Plaschkes
authors.forename: Giorgio
authors.initials: G
authors.lastname: Perilongo
authors.forename: Julia
authors.initials: J
authors.lastname: Brown
authors.forename: Jean-Bernard
authors.initials: JB
authors.lastname: Otte
authors.forename: Laurences
authors.initials: L
authors.lastname: Brugieres
authors.forename: Piotr
authors.initials: P
authors.lastname: Czauderna
authors.forename: Gordon
authors.initials: G
authors.lastname: MacKinlay
authors.forename: Anton
authors.initials: A
authors.lastname: Vos
mesh_terms: D000293:Adolescent; D000970:Antineoplastic Agents / Q000008:administration & dosage / Q000494:pharmacology*; D001706:Biopsy; D002648:Child; D002675:Child, Preschool; D002945:Cisplatin / Q000008:administration & dosage / Q000494:pharmacology*; D004317:Doxorubicin / Q000008:administration & dosage / Q000494:pharmacology*; D005260:Female; D018197:Hepatoblastoma / Q000188:drug therapy* / Q000473:pathology / Q000601:surgery*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007262:Infusions, Intravenous; D008113:Liver Neoplasms / Q000188:drug therapy* / Q000473:pathology / Q000601:surgery*; D008175:Lung Neoplasms / Q000556:secondary; D008297:Male; D009017:Morbidity; D020360:Neoadjuvant Therapy; D011183:Postoperative Complications; D011446:Prospective Studies; D013997:Time Factors; D016896:Treatment Outcome
chemical_list: D000970:Antineoplastic Agents; D004317:Doxorubicin; D002945:Cisplatin