Overview

NCI Definition: A rare neuroectodermal tumor originating from olfactory receptor cells in the nasal cavity or paranasal sinuses. Microscopically, it is characterized by neuroblastic differentiation with occasional formation of rosettes. If the tumor is not resected at an early stage, the prognosis is usually poor. [1]

Olfactory neuroblastomas most frequently harbor alterations in SUFU, IKZF1, CTCF, CDKN2C, and YES1 [2].

Most Commonly Altered Genes in Olfactory Neuroblastoma

CDKN2C Loss, YES1 Amplification, TRIM37 M556V, TP53 R306*, and TP53 Nonsense are the most common alterations in olfactory neuroblastoma [2].

Top Alterations in Olfactory Neuroblastoma

Disease Details

Synonyms
Olfactory Esthesioneuroblastoma, Head and neck esthesioneuroblastoma, NEUROBLASTOMA, OLFACTORY, MALIGNANT, Esthesioneuroblastoma, Olfactory Neuroepithelioma, Esthesioneuroepithelioma
Parent(s)
Olfactory Nerve Neoplasm
Children
Nasal Cavity Olfactory Neuroblastoma and Paranasal Sinus Olfactory Neuroblastoma
OncoTree Name
Olfactory Neuroblastoma
OncoTree Code
ONBL

References

1. National Cancer Institute. NCI Thesaurus Version 18.11d. https://ncit.nci.nih.gov/ncitbrowser/ [2018-08-28]. [2018-09-21].

2. The AACR Project GENIE Consortium. AACR Project GENIE: powering precision medicine through an international consortium. Cancer Discovery. 2017;7(8):818-831. Dataset Version 8. This dataset does not represent the totality of the genetic landscape; see paper for more information.

3. All assertions and clinical trial landscape data are curated from primary sources. You can read more about the curation process here.