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Expert Emphasizes Importance of Collaboration for Rare Pediatric Cancers

Angelica Welch
Published: Monday, Sep 10, 2018

Matthew Ladra, MD, MPH

Matthew Ladra, MD, MPH

Esthesioneuroblastoma is a rare malignancy of the paranasal sinus that occurs mostly in adults, though there is a small population of pediatric patients diagnosed annually. There is a significantly lower incidence of this disease in pediatric and adolescent patients, resulting in a lack of data and no standardization of therapy, according to Matthew Ladra, MD, MPH.

, Ladra, director, Pediatric Radiation Oncology, assistant professor, Radiation Oncology and Molecular Radiation, Johns Hopkins Kimmel Cancer Center at Sibley Memorial Hospital, discussed the treatment of patients with esthesioneuroblastoma, emphasizing the need for collaboration in this rare malignancy.

OncLive: Please provide some background information on esthesioneuroblastoma.

Ladra: This is a disease that occurs in the nasal cavity from neuronal tissue. It arises anywhere that there is olfactory epithelium. It most commonly occurs in adults, but about 20% of cases are in pediatric patients between 10 and 20 years of age. 

Being a very rare tumor, there is only about 63 cases described in the literature since 2000. Between the year 1900 and 2000, there were less than 100 cases described. Therefore, when we can get data from a multicenter collaborative effort, that is always very useful. In 2016, there was a study published with data from St. Jude Children's Research Hospital, Boston Children's Hospital, and Baylor University, which did a very good job of putting together these rare cases. They gave a summary of the outcomes for about 24 children using chemotherapy, radiation, and surgery in some combination. 

How is treatment for these patients determined?

There are a couple of stages based on the clinical presentation and how invasive it is in the nasal cavity, or what it looks like under the microscope. Based on current staging, the treatment can range from surgery alone—which is for the earliest-stage tumors—to radiation if the tumor is larger and takes up more than one side of the nasal cavity or if it looks aggressive under the microscope. 
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