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​Olfactory Neuroblastoma (Esthesioneuroblastoma)

What is Olfactory Neuroblastoma?

Olfactory neuroblastoma (also known as 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.

Olfactory neuroblastomas generally grow slowly, but in some cases may progress rapidly and aggressively. The faster growing tumors are capable of widespread metastasis.

At UPMC, the preferred surgical treatment for olfactory neuroblastomas is the Endoscopic Endonasal Approach (EEA). This innovative, minimally invasive technique uses the nose and nasal cavities as natural corridors to access hard-to-reach or previously inoperable tumors. Benefits of EEA include:

  • No incisions to heal
  • No disfigurement
  • Faster recovery time

Diagnosing Olfactory Neuroblastoma

To diagnose olfactory neuroblastoma, your doctor will:

  • Perform a physical exam
  • Ask you about any symptoms you are experiencing
  • Request imaging tests, such as MRI and CT scans, to confirm the tumor's presence

Symptoms of olfactory neuroblastoma

The most common symptom of olfactory neuroblastoma 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

Depending upon the size and extent of spread of the tumor, you may also experience:

  • Neurologic symptoms
  • Facial swelling
  • Oral and vision problems

Your doctor may refer you to a neurologist or an ophthalmologist to check for those concerns.

Olfactory Neuroblastoma Treatments

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.

Minimally invasive surgery

Olfactory neuroblastoma may be approached directly using Endoscopic Endonasal Approach (EEA). This state-of-the-art, minimally invasive approach allows surgeons to access the tumor through the natural corridor of the nose, without making an open incision. Surgeons then remove the tumor through the nose and nasal cavities.

EEA surgery offers the benefits of no incisions to heal, no disfigurement, and a faster recovery time.

EEA is an appropriate treatment option for most olfactory neuroblastomas and is advantageous as it results in fewer complications than an external approach.

If you need complementary treatments, such as radiation or chemotherapy, those therapies can begin soon after EEA surgery.

Radiation therapy and chemotherapy

Postoperative radiation therapy has been shown to lower the rate of recurrence.

Chemotherapy may be used in cases involving esthesioneuroblastomas that are recurrent, unresectable (not removable), or metastatic.

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Meet Thomas

Thomas Knox - Esthesioneuroblastoma, rare sinus cavity cancer patient story 

When Thomas developed a rare cancer in his sinus cavity, UPMC neurosurgeons acted quickly to remove the mass using the EEA technique.

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