High-dose chemotherapy followed by locoregional irradiation improves the outcome of patients with international neuroblastoma staging system Stage II and III neuroblastoma with MYCN amplification.
BACKGROUND
The objective of this study was to determine whether systemic and regional, intensified treatment can improve the outcome of children who present with a localized neuroblastoma (NB) with MYCN amplification (MNA).


METHODS
Between 1990 and 2000, 610 children with localized NB were included in the Localized Neuroblastoma 90 (NBL 90) and NBL 94 study from the French Society of Pediatric Oncology. Among them, 32 children had MNA with Stage II or III NB. During the first period of the study, 20 children (Group A) received postoperative conventional chemotherapy (CT) and/or radiotherapy (RT), depending on each patient's postoperative status. Subsequently, because of a high recurrence rate, the next 12 children (Group B) were given postoperative high-dose CT (HDC) (busulfan and melphalan) with stem cell rescue (SCR) followed by RT in addition to conventional postoperative CT.


RESULTS
The two groups were comparable with regard to prognostic factors (age, location of the primary lesion, International Neuroblastoma Staging System stage, lymph node invasion) and response to preoperative CT. The 6-year overall survival rate was significantly different between the two groups 25% +/- 10% in Group A vs. 83% +/- 11% in Group B; P = 0.004).


CONCLUSIONS
Postoperative intensification treatment with HDC, SCR, and locoregional RT resulted in higher survival rates when compared with standard treatment alone and should be considered in the treatment plan for children who are diagnosed with Stage II or III NB and MYCN amplification.
Laprie
Anne
A
Département d'Hémato-Oncologie, Hôpital des Enfants, Toulouse, France. laprie@icr.fnclcc.fr
Michon
Jean
J
Hartmann
Olivier
O
Munzer
Caroline
C
Leclair
Marc-David
MD
Coze
Carole
C
Valteau-Couanet
Dominique
D
Plantaz
Dominique
D
Carrie
Christian
C
Habrand
Jean-Louis
JL
Bergeron
Christophe
C
Chastagner
Pascal
P
Défachelles
Anne-Sophie
AS
Delattre
Olivier
O
Combaret
Valérie
V
Bénard
Jean
J
Pérel
Yves
Y
Gandemer
Virginie
V
Rubie
Hervé
H
D000293:Adolescent; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D005720:Gamma Rays; D005784:Gene Amplification; D016259:Genes, myc; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008198:Lymph Nodes / Q000473:pathology; D009361:Neoplasm Invasiveness; D009364:Neoplasm Recurrence, Local; D009367:Neoplasm Staging; D009447:Neuroblastoma / Q000188:drug therapy / Q000235:genetics* / Q000532:radiotherapy / Q000628:therapy*; D011182:Postoperative Care; D011379:Prognosis; D015996:Survival Rate; D016896:Treatment Outcome