title: Outcome of hepatoblastoma treated with the JPLT-1 (Japanese Study Group for Pediatric Liver Tumor) Protocol-1: A report from the Japanese Study Group for Pediatric Liver Tumor.
abstract: OBJECTIVE
  Hepatoblastoma is the most common malignant liver tumor in childhood. Multicenter studies elucidate the optimal pre- or postoperative chemotherapeutic regimens. This report reviews the results of the Japanese Study Group for Pediatric Liver Tumor Protocol-1 (JPLT-1) and compares its outcomes with published reports of other studies.
  
  
  METHODS
  From March 1991 to December 1999, 154 patients with malignant liver tumor including 145 cases of hepatoblastomas were enrolled in the JPLT study. Data from 134 cases were analyzed in this study. JPLT-1 protocol 91A was used for patients with stage I or II hepatoblastoma. The chemotherapy regimen consisted of repeated courses of cisplatin (CDDP), 40 mg/m(2), and tetrahydropyranyl (THP)-Adriamycin, 30 mg/m(2). JPLT-1 protocol 91B was administered to patients with stage IIIA, IIIB, or IV hepatoblastoma. The chemotherapy regimen consisted of repeated courses of CDDP, 80 mg/m(2), and THP-Adriamycin, 30 mg/m(2)/day for 2 days. Courses were repeated every 4 weeks as tolerated.
  
  
  RESULTS
  Seven patients died of chemotherapy-related side effects. Six of them died of sepsis caused by leukopenia and 1 case of liver failure. Overall survival rate (3-year/6-year) was 100%/100% for stage I (n = 9), 100%/95.7% for stage II (n = 32), 76.6%/73.8% for stage IIIA (n = 48), 50.3%/50.3% for stage IIIB (n = 25), 64.8%/38.9% for stage IV (n = 20), and 77.8%/73.4% overall. For stage IIIA and B disease, intravenous chemotherapy was better than intraarterial chemotherapy (66.4% v 38.1% for event-free survival and 69.3% v. 57.1% for overall survival). Patients less than 1 year of age had a better prognosis than older patients, but age was not a significant prognostic factor by multivariate analysis.
  
  
  CONCLUSIONS
  The overall and event-free survival rates of the JPLT-1 study of hepatoblastoma were comparable with the results of other multicenter studies in Europe and the United States. The event-free survival rate at 3 years for stage IIIB and IV disease was under 50%. New treatment strategies are needed for patients with advanced hepatoblastoma.
authors.affiliation: Department of Pediatric Surgery, Hokkaido University, School of Medicine and the JPLT, Kita-ku, Kita 15, Nishi 7, Sapporo, Japan 060-8638.
authors.forename: F
authors.initials: F
authors.lastname: Sasaki
authors.forename: T
authors.initials: T
authors.lastname: Matsunaga
authors.forename: M
authors.initials: M
authors.lastname: Iwafuchi
authors.forename: Y
authors.initials: Y
authors.lastname: Hayashi
authors.forename: H
authors.initials: H
authors.lastname: Ohkawa
authors.forename: M
authors.initials: M
authors.lastname: Ohira
authors.forename: T
authors.initials: T
authors.lastname: Okamatsu
authors.forename: T
authors.initials: T
authors.lastname: Sugito
authors.forename: Y
authors.initials: Y
authors.lastname: Tsuchida
authors.forename: A
authors.initials: A
authors.lastname: Toyosaka
authors.forename: N
authors.initials: N
authors.lastname: Nagahara
authors.forename: H
authors.initials: H
authors.lastname: Nishihira
authors.forename: Y
authors.initials: Y
authors.lastname: Hata
authors.forename: J
authors.initials: J
authors.lastname: Uchino
authors.forename: K
authors.initials: K
authors.lastname: Misugi
authors.forename: N
authors.initials: N
authors.lastname: Ohnuma
mesh_terms: D000293:Adolescent; D000367:Age Factors; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D017024:Chemotherapy, Adjuvant; D002648:Child; D002675:Child, Preschool; D002945:Cisplatin / Q000008:administration & dosage; D004317:Doxorubicin / Q000008:administration & dosage / Q000031:analogs & derivatives*; D005260:Female; D018197:Hepatoblastoma / Q000188:drug therapy* / Q000401:mortality / Q000473:pathology / Q000601:surgery; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007269:Injections, Intra-Arterial; D007275:Injections, Intravenous; D008113:Liver Neoplasms / Q000188:drug therapy* / Q000401:mortality / Q000473:pathology / Q000601:surgery; D008297:Male; D009367:Neoplasm Staging; D015996:Survival Rate; D016896:Treatment Outcome
chemical_list: D004317:Doxorubicin; C027260:pirarubicin; D002945:Cisplatin