Dramatic Response to Sorafenib in Olfactory Neuroblastoma

Background: Olfactory neuroblastomas (ONBs) are rare nasal sinus tumors that arise from the olfactory epithelium. Their management requires multimodal therapy that can consist of surgery, radiotherapy, and chemotherapy. We present a case of an ONB with extensive cranial invasion that demonstrated dramatic response to sorafenib, a tyrosine kinase inhibitor, and review the literature on management with targeted inhibitors.

Case: A 54-year old man with history of prostate cancer and melanoma presented with left proptosis and rhinorrhea. CT and PET revealed a 6.5 cm, Kadish Stage D ONB with extensive cranial invasion. It was refractory to traditional chemotherapy but demonstrated dramatic response to sorafenib, causing extensive skull base defects that prompted surgical repair. The patient remains radiologically and clinically stable 34 months after resection.

Discussion: We review the clinical presentation and multimodal management of a large ONB with dramatic response to the tyrosine kinase inhibitor sorafenib. We also highlight prior uses of targeted therapy in the management of refractory ONB within the context of current standard treatment regiments. Targeted therapies may play a vital role in the management of refractory ONB. Our report adds to the sparse literature on the use of targeted therapies as salvage therapy for olfactory neuroblastoma.