Esthesioneuroblastoma is a very rare cancer that develops in the upper part of the nasal cavity. It is thought to arise from neural tissue associated with the sense of smell. This tumor generally grows slowly but in some cases may progress rapidly and aggressively. The faster growing tumors are capable of widespread metastasis.
The most common symptom of esthesioneuroblastoma is chronic congestion on one side of the nose.
The preferred surgical treatment at UPMC for esthesioneuroblastoma is the Endoscopic Endonasal Approach (EEA) to remove the tumor. This innovative, minimally invasive technique uses the nose and nasal cavities as natural corridors to access hard-to-reach or previously inoperable tumors. EEA offers the benefits of no incisions to heal, no disfigurement to the patient, and a faster recovery time. If complementary treatments, such as chemotherapy or radiation therapy, are needed, those therapies can begin soon after surgery.
Your physician will perform a physical exam and ask you about any symptoms you are experiencing. The most common symptom of esthesioneuroblastoma is chronic congestion on one side of the nose. This may continue for months or even years before a diagnosis is made. Other symptoms may include:
- nasal discharge
- sinusitis
- loss of the sense of smell
The size and extent of spread of the tumor may cause neurologic symptoms, facial swelling, and oral and vision problems. Your physician also may refer you to a neurologist or an ophthalmologist to check for those concerns.
Your physician may want you to have imaging procedures, such as MRI and CT scans, to confirm the tumor's presence.
Surgery, radiation therapy, or a combination of the two are typically used to treat these tumors. Esthesioneuroblastomas often recur locally and may also spread to other parts of the body. Treatment that is early and aggressive produces the best prognosis.
Esthesioneuroblastomas of the skull base may be approached directly by using the Endoscopic Endonasal Approach (EEA). This approach allows surgeons to see the tumor well without making incisions in the face or skull. The tumor is removed through the nose and nasal cavities, and recovery time is faster than with a traditional approach. EEA is appropriate for most esthesioneuroblastomas and is advantageous as it results in fewer complications than an external approach.
At UPMC, we take a 360° Approach to treatment when evaluating each patient—looking at their conditions from every direction—to find the path that is least disruptive to the patient's brain, critical nerves, and ability to return to normal functioning. Our neurosurgical team may recommend a combination of surgical and non-surgical approaches to maximize the benefits of surgery while minimizing risks.
Other Treatments
Patients who received surgery with the Endoscopic Endonasal Approach may be able to begin chemotherapy or radiation therapy soon after surgery.
Postoperative radiation therapy has been shown to lower the rate of recurrence. Chemotherapy may be used in cases involving recurrent, unresectable, or metastatic esthesioneuroblastoma.