Results of salvage therapy after failure of initial treatment for advanced olfactory neuroblastoma

Abstract

Olfactory neuroblastoma is a very aggressive tumour with high rates of locoregional recurrence and distant metastasis. Therefore, salvage therapy plays an important role in control of these neoplasms. In this study, we present our experience and treatment outcomes for salvage therapy in patients with recurrent olfactory neuroblastoma. We retrospectively analysed 17 patients treated for advanced olfactory neuroblastoma during the last 15 years. The disease-free 5-year survival rate was 48% in all patients and 42% in the 17 patients with recurrence or distant metastasis. Salvage therapy was performed in 6 of 8 patients with recurrence or metastasis and proved successful in 50% of them. In the patients with locoregional recurrence, the success rate of salvage radiotherapy plus neck dissection was superior to salvage chemotherapy alone. In addition, 17% of the patients with distant metastasis after initial treatment died after salvage therapy. In 15% of patients with a clinical stage N0 at initial diagnosis, nodal recurrence developed and was successfully treated with salvage therapy. Complete surgical resection, including craniofacial resection and postoperative radiotherapy without elective neck dissection, is the preferred approach in the treatment of advanced olfactory neuroblastoma. In locoregional recurrence, successful salvage therapy may include selective neck dissection and radiotherapy, but in cases of distant metastasis, the prognosis was poor.