title: Long-term survivors of advanced neuroblastoma with MYCN amplification: A report of 19 patients surviving disease-free for more than 66 months.
abstract: OBJECTIVE
  According to initial reports, stage 4 neuroblastoma patients with amplification of the MYCN proto-oncogene developed progressive disease within 8 months. The prognosis for such patients, however, should now be reevaluated in light of recent results achieved with up-to-date combination chemotherapy.
  
  
  METHODS
  Patients with stage 3, 4, and 4S neuroblastoma and more than 10 copies of MYCN received induction chemotherapy, which from January 1985 to February 1991 consisted of regimen A(1 )(cyclophosphamide 1,200 mg/m(2) on day 1, vincristine 1.5 mg/m(2) on day 1, pirarubicin 40 mg/m(2) on day 3, and cisplatin 90 mg/m(2) on day 5) and from March 1991 to September 1993 consisted of regimen A(3 )(cyclophosphamide 1,200 mg/m(2) on days 1 and 2, pirarubicin 40 mg/m(2) on day 3, etoposide 100 mg/m(2) on days 1 through 5, and continuous infusion cisplatin 25 mg/m(2) on days 1 through 5). Most of these patients underwent radical surgery to remove the original tumor and local metastases, irradiation, and supralethal preconditioning regimens, followed by blood stem-cell transplantation (SCT). Data on the patients were collected in December 1998, and the factors contributing to disease-free survival were analyzed.
  
  
  RESULTS
  During the study period, 66 patients with more than 10 copies of MYCN were treated. Five of nine patients with stage 3 disease, 13 of 55 with stage 4, and one of two with stage 4S survived for at least 66 months. It is interesting that all but one patient who survived for more than 66 months underwent SCT, in contrast with only five of 45 patients who died.
  
  
  CONCLUSIONS
  Not all patients with advanced neuroblastoma who have more than 10 copies of MYCN will die. The requisites for survival in such patients seem to be intensive induction chemotherapy, effective surgery, irradiation, and the use of SCT.
authors.affiliation: Study Group of Japan for Treatment of Advanced Neuroblastoma, Izumi, Japan.
authors.forename: K
authors.initials: K
authors.lastname: Kawa
authors.forename: N
authors.initials: N
authors.lastname: Ohnuma
authors.forename: M
authors.initials: M
authors.lastname: Kaneko
authors.forename: K
authors.initials: K
authors.lastname: Yamamoto
authors.forename: T
authors.initials: T
authors.lastname: Etoh
authors.forename: H
authors.initials: H
authors.lastname: Mugishima
authors.forename: M
authors.initials: M
authors.lastname: Ohhira
authors.forename: J
authors.initials: J
authors.lastname: Yokoyama
authors.forename: F
authors.initials: F
authors.lastname: Bessho
authors.forename: T
authors.initials: T
authors.lastname: Honna
authors.forename: J
authors.initials: J
authors.lastname: Yoshizawa
authors.forename: K
authors.initials: K
authors.lastname: Nakada
authors.forename: M
authors.initials: M
authors.lastname: Iwafuchi
authors.forename: T
authors.initials: T
authors.lastname: Nozaki
authors.forename: J
authors.initials: J
authors.lastname: Mimaya
authors.forename: T
authors.initials: T
authors.lastname: Sawada
authors.forename: T
authors.initials: T
authors.lastname: Nakamura
authors.forename: H
authors.initials: H
authors.lastname: Miyata
authors.forename: K
authors.initials: K
authors.lastname: Yamato
authors.forename: Y
authors.initials: Y
authors.lastname: Tsuchida
mesh_terms: D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D002675:Child, Preschool; D002945:Cisplatin / Q000008:administration & dosage; D003520:Cyclophosphamide / Q000008:administration & dosage; D004273:DNA, Neoplasm / Q000032:analysis; D018572:Disease-Free Survival; D004317:Doxorubicin / Q000008:administration & dosage / Q000031:analogs & derivatives; D005047:Etoposide / Q000008:administration & dosage; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D008297:Male; D009447:Neuroblastoma / Q000188:drug therapy / Q000235:genetics* / Q000473:pathology; D011379:Prognosis; D000090063:Proto-Oncogene Mas; D016271:Proto-Oncogene Proteins c-myc / Q000235:genetics*; D017741:Survivors; D014750:Vincristine / Q000008:administration & dosage
chemical_list: D004273:DNA, Neoplasm; C000715470:MAS1 protein, human; D000090063:Proto-Oncogene Mas; D016271:Proto-Oncogene Proteins c-myc; D014750:Vincristine; D005047:Etoposide; D004317:Doxorubicin; D003520:Cyclophosphamide; C027260:pirarubicin; D002945:Cisplatin