Olfactory neuroblastoma / Esthesioneuroblastoma

Symptoms are those common in any sinonasal malignancy: unilateral nosebleeds, nasal airway obstruction, and sometimes pain. The diagnosis may be strongly suggested by a CT or MRI done to evaluate such symptoms, but a tissue diagnosis in necessary for diagnosis.

Diagnosis requires a biopsy, reviewed by an experienced patholologist. Although often a CT is done in initial evaluation, and MRI is necessary to evaluate the interface between the top of the ethmoid sinus and the brain, as well as to be sure the orbit is uninvolved. Spread to other sites is uncommon, but the neck should be evaluated by imaging as about 15% of patients develop involved neck nodes over time.

Treatment depends largely on anatomic extent of disease. Most olfactory neuroblastomas have mot metastasized and are resectable. Hence local treatment is key. Most agree resection is the initial treatment of choice, and  most agree that postoperative irradiation with intensity-modulated radiation therapy (IMRT) should follow. Today endoscopic approaches are usually sufficient to resect the tumor and repair at the same time the skull base. An open craniotomy is occasionally needed for selected larger tumors. Chemotherapy is generally reserved for advanced tumors that involve the brain or orbit, or for recurrence. If there are involved neck nodes they are removed at the time of removal of the sinonasal tumor or soon thereafter. Most patients with an esthesioneuroblastoma are cured: the 5-year local control rate is > 75%, possibly 90 to 95%. Although metastases are uncommon at presentation, ~10 to 15% will develop cervical nodal metastases. Distant metastases do occur rarely, and can be seen as late as a decade following the initial intervention. Late recurrences can be along dura as late as 15 years after surgery and are amenable to stereotactic radiosurgery. Long-term follow-up hence is necessary.