Esthesioneuroblastoma (Olfactory Neuroblastoma)

Esthesioneuroblastoma (also known as Olfactory Neuroblastoma) accounts for 2-6% of nasal and sinus cavity tumors. The cell origin for this malignant tumor is the olfactory neuroepithelium in the superior nasal cavity along the superior turbinate and cribriform plate (thin bone at the roof of the nose and sinuses separating the brain from the nasal and sinus cavities). Patients with esthesioneuroblastoma often present to the doctor with complaints of one-sided nasal blockage and nose bleeds. Specifically, 70% of patients with esthesioneuroblastoma have unilateral nasal obstruction and 50% have nose bleeds when they seek medical attention. Esthesioneuroblastoma is stratified into different stages by the Kadish Staging System.

Kadish Staging:

A: Tumor is limited to the nasal cavity
B: Tumor is limited to the nasal cavity and sinuses
C: Tumor is found invading the cribriform plate, skull base or intracranial cavity
D: Tumor has spread via the lymph nodes or blood stream to regional or distant sites

Treatment of Esthesioneuroblastoma:

Surgery combined with radiation therapy (and in some cases, chemotherapy) is the standard of care for esthesioneuroblastoma. Patients with this malignant tumor are best served by having an evaluation with an otolaryngologist (ENT physician), neurosurgeon, radiation oncologist and possibly medical oncologist.

There are two main approaches for surgical removal of esthesioneuroblastoma. One approach, craniofacial resection involves incisions on the scalp and/or face to approach the tumor and expose the adjacent brain through the outside of the skull. The second approach, endoscopic trans-nasal resection, involves using endoscopes and specialized instruments to work through the nasal passages to remove the tumor from underneath the brain, inside the skull. Most studies report similar cancer control rates with both surgical approaches. (1-5) Generally speaking the endoscopic approach through the nose is most commonly applied to smaller tumors (Kadish Stage A & B) while craniofacial resection is most commonly applied to larger, more invasive tumors (Kadish Stage C & D). After treatment, close follow up with an otolaryngologist and neurosurgeon is recommended to monitor for signs of tumor recurrence.

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References:

1. Lund VJ, Stammberger H, Nicolai P et al. Rhinology 2010;1:146-51.
2. Stammberger H, Anderhuber W, Walch C. Acta Otolaryngol Belg 1999; 53:199-205.
3. Walch C, Stammberger H, Anderhuber W. Laryngoscope 2000; 110:635-640
4. Devaiah AK, Andreoli MT. T Laryngoscope 2009; 119:1412-1416.
5. Castelnuovo PG, Delu G, Sberze E et al. Skull Base 2006; 16:25-30.