ESTHESIONEUROBLASTOMA

Olfactory Neuroblastoma; Skull Based Olfactory Neuroblastoma; Intracranial Olfactory Neuroblastoma; Recurrent Esthesioneuroblastoma

Esthesioneuroblastoma is a rare cancer of the upper part of the nasal cavity called the cribriform plate, which is a bone deep in the skull between the eyes, and above the ethmoid sinuses. It develops in nerve tissue associated with the sense of smell and can occur in people of any age. This cancer is very uncommon, accounting for 7 percent of all cancers of the nasal cavity and paranasal sinuses. Although it generally grows slowly, an esthesioneuroblastoma can sometimes grow very quickly. Fast-growing tumors can metastasize (spread) even many years after treatment of the initial tumor.

Diagnostic testing:

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  Physical examination of the eyes, nose and throat;

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  Endoscopy examination of the mouth and nose;

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  CT, MRI, or PET scan;

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  Biopsy; or

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  Laboratory testing of the blood and urine.

Physical findings: People with an esthesioneuroblastoma may present with:

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  Nasal obstruction;

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  Nosebleeds (epistaxis);

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  Changes in the sense of smell (hyposmia);

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  Nasal discharge (pus);

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  Facial pain;

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  Changes in vision;

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  Excessive tearing from the eyes (lacrimation);

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  Facial or tooth numbness;

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  Ear pain or pressure;

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  Enlarged lymph nodes in the neck; and

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  Difficulty opening the mouth.

ICD-9: 160.0

Esthesioneuroblastoma usually begins in the part of the brain that interprets smell (olfactory bulb). This tumor may occur at any age, and tends to form behind the nose and may affect the sinuses. People with olfactory neuroblastomas may lose the sense of smell, have frequent nosebleeds, and may experience difficulties breathing through their nose.

Esthesioneuroblastomas are treated with surgery, radiation, and chemotherapy depending on the response to treatment and metastasis. These treatments may affect eating, speaking, or breathing.

SUGGESTED PROGRAMMATIC ASSESSMENT*

Suggested MER for Evaluation:

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  Clinical history and examination that describes the diagnostic features of the impairment;

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  Endoscopy reports;

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  CT/MRI and PET scan reports; and

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  Biopsy reports.

Suggested Listings for Evaluation:

REMARKS

Must be progressive or recurrent following initial antineoplastic therapy.

Metastatic disease medically equals the criteria in listing 13.13 B 1. (Kadish classification system Stage D).