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Localized Pelvic Neuroblastoma: Excellent Survival and Low Morbidity With Tailored Therapy—The 10-Year Experience of the French Society of Pediatric Oncology

From the Service de Chirurgie Infantile and Service d'Oncologie Pédiatrique, Hôpital Mère-Enfant, CHU de Nantes, Nantes; Département de Pédiatrie, Institut Gustave Roussy, Villejuif; Service de Chirurgie Pédiatrique, CHRU Dupuytren, Limoges; Département de Radiothérapie, Institut Gustave Regaud; and Unité d'Hémato-oncologie, Hôpital des enfants, Toulouse, France

Address reprint requests to Marc-David Leclair, MD, Service de Chirurgie Infantile, Hôpital Mère-Enfant, CHU de Nantes, 44093 Nantes Cedex 01, France; e-mail: marcdavid.leclair{at}chu-nantes.fr

PURPOSE: To assess the results and morbidity of treatment of children with localized pelvic neuroblastoma (NB).

PATIENTS AND 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).

CONCLUSION: 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.

Supported by the Association for Cancer Research and the Clinical Research Program of the French Ministry of Health (grant No. [3]95-06-N).

Presented in part at the 13th Annual Meeting of the European Society for Pediatric Urology, April 2002, Budapest, Hungary.

Authors' disclosures of potential conflicts of interest are found at the end of this article.

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