Olfactory Neuroblastoma

Olfactory neuroblastoma, also called esthesioneuroblastoma, is a type of skull base tumor. It usually grows in the top part of the nasal cavity, close to the base of the brain and bones around the eyes. It can also spread to other parts of the body.

Olfactory Neuroblastoma Symptoms

Common symptoms include:

• Nose bleeds
• Congestion
• Loss of smell
• Difficulty breathing through the nose
• Lump in the neck
• Head or facial pain
• Vision changes

Because olfactory neuroblastoma tumors grow near important structures like the brain, nose, eyes, and facial nerves, your care team should include multiple specialists. At Duke, a head and neck surgeon and/or sinus specialist serves as your primary doctor. Neurosurgeons, oncologists, neuro-ophthalmologists, plastic surgeons, and others may also be involved in your care as needed.

Long-Term Follow-Up
Olfactory neuroblastomas can recur many years after successful treatment. It’s important to seek care at a center like Duke, where our specialists offer quality, long-term monitoring for this and other types of rare cancers.

Imaging Scans 
X-rays, CT scans, and/or MRIs help doctors identify your tumor’s location and size.

PET Scan
A radioactive dye, which is injected intravenously, concentrates in olfactory neuroblastoma tumors. This enables imaging specialists to accurately pinpoint the tumor's location, assess the cancer's stage, and determine whether it has spread to other parts of the body. Duke offers the "gallium-68 dotatate" PET scan, which specifically targets esthesioneuroblastoma cells, allowing for the most precise diagnosis and staging results.

Biopsy
A doctor inserts specialized tools through your nostrils to remove a small portion of the tumor. Certain tumors may require surgery to extract a biopsy sample. A pathologist then examines the tissue under a microscope to determine the tumor type and its growth rate. Duke pathologists who specialize in these rare cancers help ensure an accurate diagnosis.

Surgery to remove the tumor combined with radiation therapy is the standard treatment for olfactory neuroblastoma. Your care team may also recommend chemotherapy before or after surgery.

Surgery
Duke surgeons offer several approaches to safely remove olfactory neuroblastoma tumors. Surgeons may also remove nearby lymph nodes in the head and neck if the cancer has spread.

• Endoscopic endonasal: For tumors that are not deep within the skull, surgeons may use a camera called an endoscope to operate through the nostrils and remove the tumor. This minimally invasive technique does not require any external incisions, speeding recovery time and resulting in no external scarring or brain retraction (pulling back brain tissue). This technique is also called transnasal endoscopic surgery.
   
• Transorbital endoscopic / multi-portal: This technique combines the endoscopic endonasal approach with a small incision beneath the eyebrow, enabling surgeons to access the nasal cavity through the eye socket. Less invasive than a craniotomy, the transorbital endoscopic approach is best-suited for people whose tumors have invaded the eye socket or are more easily reached through it, helping to avoid brain retraction.
   
• Transfacial: This approach is typically reserved for larger tumors that cannot be safely removed through the nostrils using an endoscope. A head and neck surgeon makes a flap-like incision below the eye, along the side of the nose, and above the upper lip. The tumor is then carefully removed, and the incision is closed to minimize scarring. Like the endonasal and transorbital approaches, this technique reduces interaction with the brain and lowers the risk of complications.
   
• Craniotomy: For larger or more complex tumors that have invaded the brain, surgeons remove a small piece of the skull to reach and remove the tumor.

Radiation Therapy
After the tumor is surgically removed, radiation therapy applies energy rays to kill remaining cancer cells. Duke offers intensity-modulated radiation therapy (IMRT), which customizes the intensity and shape of the rays to target precise areas and spare surrounding healthy tissue.

Chemotherapy
Chemotherapy may be recommended before surgery to shrink the tumor, or after surgery to treat cancer that has spread to other parts of the body.

Patient Navigators Coordinate Your Care
Our patient navigator guides you through every step of your treatment. They help schedule appointments, coordinate visits, communicate your test results, and prepare you for surgery. And they are available to answer all your questions along the way.

Innovative Research Informs Care
Due to its rarity, olfactory neuroblastoma is not researched as often as other cancers. However, Duke specialists who focus on nasal and sinus cancers are among the few working to develop advanced treatments for olfactory neuroblastoma, identify target molecules for precision drug therapies, and better predict prognosis.