Esthesioneuroblastoma in childhood and adolescence. Better prognosis with multimodal treatment?

Abstract

Background and purpose: Only 3% of all malignant intranasal tumors are esthesioneuroblastomas (ENB) and only 20% of these rare neuroectodermal tumors are diagnosed up to 20 years of age. Radiotherapy and surgery are established treatment modalities for these patients, but the role of chemotherapy, especially in a multimodal approach, is not well defined. To investigate the influence of radio- and chemotherapy, the treatment and course of the disease in children and adolescents with ENB were analyzed retrospectively.

Patients and methods: 19 unselected patients (nine male and ten female) diagnosed with ENB < or = 20 years of age were included in this analysis. Median age at diagnosis was 14.0 years (range, 5-20 years). The tumors were Kadish stage B in 4/19 patients and stage C in 15/19 patients. 17 patients underwent surgery, either without further therapy (n = 4), followed by radiotherapy (n = 1) or as part of multimodal regimens (n = 12). Two patients received radio- and chemotherapy without surgery. Complete resection (R0) was achieved in 15 out of 17 patients with surgery including all five patients with preoperative chemotherapy due to unresectable primary at diagnosis.

Results: The 5-year overall survival (OS) for the whole group was 73% +/- 12% and the 5-year event-free survival (EFS) 55% +/- 13%. None of the four patients with stage B experienced tumor progression so far, whereas seven out of 15 patients with stage C did (5-year EFS 47% +/- 14%; not significant). Patients with Kadish stage C and multimodal treatment strategies combing surgery, chemo- and radiotherapy had a significantly better outcome than patients with stage C and less than three treatment modalities (65% +/- 17% vs. 20% +/- 18%; p = 0.02).

Conclusion: These data indicate a benefit of multimodal treatment regimens combining surgery, chemo- and radiotherapy for pediatric patients with ENB Kadish stage C. Chemotherapy appears to improve resectability, EFS, and OS. Radiotherapy is an integral part in the management of children and young adolescents with ENB in Kadish stage B and C.