Olfactory neuroblastoma is a rare and aggressive tumor of the nasal cavity. It is a type of neuroendocrine tumor that arises from the olfactory nerve in the upper nasal cavity. It is thought to be caused by a mutation in the RET proto-oncogene, however the exact cause is unknown.
Olfactory neuroblastoma is typically diagnosed with a combination of imaging studies and histology. CT or MRI scans of the head and neck are usually used to detect the tumor. Endoscopic biopsy is then used to obtain tissue for histological analysis.
Other tumors of the nasal cavity such as adenoid cystic carcinoma, esthesioneuroblastoma, and inverted papilloma must be ruled out when diagnosing olfactory neuroblastoma.
Treatment for olfactory neuroblastoma is typically a combination of surgery and radiation therapy. Chemotherapy may also be used for more advanced cases.
The prognosis for olfactory neuroblastoma is generally poor. The 5-year survival rate is approximately 30-50% depending on the stage of the tumor at diagnosis.