Esthesioneuroblastoma

Orpha code: 1957OMIM code:

Definition

Esthesioneuroblastoma (ENB) is a rare malignant neoplasm of the sinonasal cavity, arising from the basal layers of olfactory neuroepithelial cells in the superior nasal vault, which usually occurs in the 5th to 6th decades of life and is characterized clinically by non-specific symptoms such as progressive ipsilateral nasal block, sinusitis, facial pain, intermittent headaches, hyposmia/dysosmia, rhinorrhea and epistaxis as well as proptosis, diplopia and excessive lacrimation due to orbital extension. With early treatment and in the absence of distant metastases, ENB appears to have a good prognosis (compared to other superior nasal malignancies), despite a high rate of cervical metastases.

Disease data
Classification

Disease

Synonyms
Olfactory neuroblastoma
Nerwiak węchowy zarodkowy
ORPHA code
1957
OMIM code
-
ICD10 code
C30.0
ICD11 code
2C20.3

No additional description.

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