ESTHESIONEUROBLASTOMA
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ALTERNATE NAMES
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Olfactory Neuroblastoma; Skull Based Olfactory Neuroblastoma; Intracranial Olfactory
Neuroblastoma; Recurrent Esthesioneuroblastoma
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DESCRIPTION
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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.
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DIAGNOSTIC TESTING, PHYSICAL FINDINGS, AND
ICD-9-CM/ICD-10-CM
CODING
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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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Laboratory testing of the blood and urine.
Physical findings: People with an esthesioneuroblastoma may present with:
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Changes in the sense of smell (hyposmia);
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Excessive tearing from the eyes (lacrimation);
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Facial or tooth numbness;
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Enlarged lymph nodes in the neck; and
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Difficulty opening the mouth.
ICD-9: 160.0
ICD-10: C30.0
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PROGRESSION
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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.
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TREATMENT
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Esthesioneuroblastomas are treated with surgery, radiation, and chemotherapy depending
on the response to treatment and metastasis. These treatments may affect eating, speaking,
or breathing.
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SUGGESTED PROGRAMMATIC ASSESSMENT*
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Suggested MER for
Evaluation:
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Clinical history and examination that describes the diagnostic features of the impairment;
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CT/MRI and PET scan reports; and
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Suggested Listings for Evaluation:
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DETERMINATION
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LISTING
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REMARKS
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Meets
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13.13 A 2
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Must be progressive or recurrent following initial antineoplastic therapy.
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113.21 A, B, C, or D
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Must either: extend across the midline, have distant metastases, be recurrent, or
have onset at age 1 year or older.
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Equals
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13.13 B 1
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Metastatic disease medically equals the criteria in listing 13.13 B 1. (Kadish classification
system Stage D).
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* Adjudicators may, at their discretion, use the Medical Evidence of Record or the
listings suggested to evaluate the claim. However, the decision to allow or deny the
claim rests with the adjudicator.
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