Olfactory Neuroblastoma (ONBL)

Overview

Olfactory Neuroblastoma (also called esthesioneuroblastoma) is a rare malignant neuroectodermal tumor arising from olfactory epithelium in the sinonasal region. It sits under the Embryonal Tumor (EMBT) branch in OncoTree. Clinical behavior is highly variable; NGS profiling can reveal actionable alterations in advanced cases.

Cohorts in the corpus

• hnc_mskcc_2016 — 2 olfactory neuroblastoma cases among 151 recurrent/metastatic head and neck tumors profiled by MSK-IMPACT 410-gene panel PMID:27442865.

Recurrent alterations

• Specific alteration frequencies not reported for the 2-patient ONBL subset; both cases contributed to the overall basket-trial enrollment framework where 21% of 135 advanced head and neck tumors harbored potentially actionable alterations PMID:27442865.

Subtypes

• Not further subclassified in the corpus.

Therapeutic landscape

• As part of the MSK-IMPACT cohort, ONBL patients with actionable alterations were candidates for basket-trial enrollment PMID:27442865.

Sources

• PMID:27442865 — Morris et al. 2017 (JAMA Oncol). 2 ONBL cases in MSK-IMPACT advanced head and neck cohort.

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