Localized pelvic neuroblastoma: excellent survival and low morbidity with tailored therapy--the 10-year experience of the French Society of Pediatric Oncology.
OBJECTIVE
To assess the results and morbidity of treatment of children with localized pelvic neuroblastoma (NB).


METHODS
All consecutive cases of localized pelvic NB registered in the French multicenter prospective studies NBL90 and NBL94 between 1990 and 1999 were reviewed. Resectability was decided on the basis of clinical and radiologic evaluation. In unresectable tumors, primary chemotherapy (combinations of carboplatin-etoposide and vincristine-cyclophosphamide-doxorubicine) was administered before surgery.


RESULTS
Forty-seven children (with 26 resectable tumors and 21 unresectable) were included in this study. At the end of treatment, 31 children were in complete remission (66%). Long-term neurologic sequelae were observed in seven patients (15%), directly attributable to surgery in three cases. After a median follow-up of 48 months (range, 13 to 129 months), 44 patients are alive. Six children experienced local relapse; four of these children achieved subsequent remission. The projected overall survival and event-free survival (EFS) rates at 5 years are, respectively, 93% +/- 4% and 84% +/- 5%. Survival of children treated with preoperative chemotherapy are similar to those treated by primary surgery (80% and 88% respectively). The extent of surgical resection seemed to have no influence on the outcome (EFS rates 76% and 89% in case of gross residue and complete resection or microscopic residue, respectively).


CONCLUSIONS
Our data confirm the excellent survival of localized pelvic NBs. Considering the efficacy of preoperative chemotherapy, patients with pelvic NB should be carefully screened for primary surgery. The risk of neurologic impairment during radical excision should be balanced with the good survival of children with minimal residual disease.
Leclair
Marc-David
MD
Service de Chirurgie Infantile, Hôpital Mère-Enfant, CHU de Nantes, 44093 Nantes Cedex 01, France. marcdavid.leclair@chu-nantes.fr
Hartmann
Olivier
O
Heloury
Yves
Y
Fourcade
Laurent
L
Laprie
Anne
A
Mechinaud
Françoise
F
Munzer
Caroline
C
Rubie
Herve
H
D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D009017:Morbidity; D020360:Neoadjuvant Therapy; D009364:Neoplasm Recurrence, Local; D009447:Neuroblastoma / Q000188:drug therapy* / Q000473:pathology / Q000601:surgery*; D010386:Pelvic Neoplasms / Q000188:drug therapy* / Q000473:pathology / Q000601:surgery*; D011183:Postoperative Complications; D011379:Prognosis; D012189:Retrospective Studies; D016019:Survival Analysis