[1] Due to the location of the tumor and its proximity to the cranial cavity, esthesioneuroblastoma can be highly invasive and challenging to treat.

Of the research to date, the sonic hedgehog pathway, MYC and KDR genes are implicated for esthesioneuroblastoma.

[12] Esthesioneuroblastoma is characterized by neurofibrillary stroma and neurosecretary granules that are not seen concurrently by any other pathologies in the region.

[1] Histological tests such as keratin, CK5/6, S-100 protein or NSE can be run to further differentiate esthesioneuroblastoma from other tumors.

For cases with cribriform plate involvement, tumors are resected bilaterally using a transfacial and craniotomy approach.

[14][21] Proton radiotherapy has recently been shown to be effective in a 10-person study with Kadish C tumors, while delivering less toxicity to the nervous system.

[4] Cyclophosphamide, vincristine and doxorubicin have been used as neoadjuvant chemotherapy drugs for grade C esthesioneuroblastoma before surgical resection, producing fair outcomes.

In advanced stage esthesioneuroblastoma in pediatric patients, where surgery is no longer possible, aggressive chemotherapy and radiotherapy has resulted in some tumor control and long-term survival.

[25] Esthesioneuroblastoma is a slow developing but malignant tumor with high recurrence rates because of its anatomical position.

[citation needed] Esthesioneuroblastoma accounts for 2% of all intranasal tumors with an incidence of 0.4 cases per million people.

[4][1] Esthesioneuroblastoma can occur at any time, with peak occurrence reported in the second and sixth decades of life.