[Comprehensive protocol for diagnosis and treatment of childhood neuroblastoma--results of 45 cases].
OBJECTIVE
The aim of the paper was to improve the prognosis of neuroblastoma (NB) stage III and IV in children through the comprehensive therapy including chemotherapy, delayed tumor resection, autologous stem cell transplantation (ASCT) and inducing differentiation and to analyze the factors affecting the prognosis.


METHODS
Newly diagnosed neuroblastoma patients seen from Oct.1998 to Dec.2003 were divided into high, medium and low risk groups depending on clinical stage and age. Comprehensive protocol included accurate staging, delayed and/or second tumor resection for stage III and IV patients, chemotherapy of different intensity mainly composed of cell cycle nonspecific drugs and 13-cis-retinoid for inducing cell differentiation. ASCT was given at the end of therapy for high risk group.


RESULTS
Forty-five patients, 6 months to 11 years of age, 32 males and 13 females, were analyzed. Of them, 15 were found to have the tumor in adrenal gland, 12 had the tumor extended to the retro-peritoneal space, while in 15 cases the tumor was beside the spinal column in chest and in 3 the tumor was located in other places. Nine cases had stage I, 1 case had stage II, 8 cases had III, 26 cases had stage IV and 1 case had stage IVs of the tumor. Depending on the age and stage of the tumor, 26 cases were aligned into high risk protocol, 10 into medium risk and 9 into low risk groups. Thirty nine cases were treated as planned. Eleven of them received ASCT including 2 cases who received second ASCT. Of the thirty-nine patients, 31 achieved complete remission (CR) and 8 partial remission (PR) after surgery and/or chemotherapy. During up to 21 months median following up period (range 14 to 64 months), 24 cases (62%) kept CR (median 22 months) and 4 survived with stable disease. The survival rate (SR) was 72%. Eleven cases died of relapse and disease progression. No death occurred from treatment complication. Statistical analysis showed that the age older than 18 months, and stage III and IV of the tumor were the factors predicting poor prognosis (P = 0.04 and 0.003, respectively). Patients who had the tumor originated from the retroperitoneal space, who had incomplete tumor resection, and those who did not receive ASCT had poorer prognosis, but the differences were not significant (P = 0.092, 0.55 and 0.60, respectively).


CONCLUSIONS
The comprehensive protocol seemed to be reasonable. Age older than 18 months, and stage III and IV were the factors suggesting poor prognosis. The origin of the tumor, completeness of tumor resection, and use of ASCT had no significant impact on the prognosis.
Tang
Jing-yan
JY
Department of Hematology/Oncology, Shanghai Children's Medical Center, Shanghai Jiaotong University Medical College, Shanghai 200127, China.
Pan
Ci
C
Chen
Jing
J
Xu
Min
M
Chen
Jing
J
Xue
Hui-liang
HL
Gu
Long-jun
LJ
Dong
Ru
R
Ye
Hui
H
Zhou
Min
M
Wang
Yao-ping
YP
D000367:Age Factors; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D017024:Chemotherapy, Adjuvant; D002648:Child; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D018380:Hematopoietic Stem Cell Transplantation / Q000379:methods; D006801:Humans; D007223:Infant; D008297:Male; D009364:Neoplasm Recurrence, Local / Q000517:prevention & control / Q000601:surgery / Q000628:therapy; D009367:Neoplasm Staging; D009447:Neuroblastoma / Q000175:diagnosis* / Q000188:drug therapy / Q000401:mortality / Q000473:pathology / Q000601:surgery / Q000628:therapy*; D011379:Prognosis; D012074:Remission Induction / Q000379:methods; D012189:Retrospective Studies; D012720:Severity of Illness Index; D015996:Survival Rate; D013997:Time Factors; D014182:Transplantation, Autologous / Q000379:methods; D016896:Treatment Outcome