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Where does esthesioneuroblastoma develop?

Esthesioneuroblastoma can develop in several places.

• Olfactory bulb: The olfactory bulb is considered part of the brain and located above the nasal cavity.
• Olfactory nerves: These are the nerves that control your sense of smell.
• Olfactory receptors: This is the area at the roof of the nose that detects various odors and aromas for us to be able to smell.

Esthesioneuroblastoma can spread to the sinuses, eyes and brain. It can also spread to other parts of the body, such as the lymph nodes in your neck.

What are the symptoms of esthesioneuroblastoma?

Esthesioneuroblastoma symptoms may include:

• Loss of sense of smell
• Nasal blockage

As esthesioneuroblastoma grows, advanced symptoms can include:

• Nosebleeds
• Facial pain and pressure
• Recurrent sinus infections
• Vision problems

When to see a doctor for symptoms

Esthesioneuroblastoma symptoms can mimic symptoms of other common benign conditions like allergies, colds and sinus infections. So, pay attention to how long you¡¯ve had your symptoms and if they¡¯re affecting one or both sides of your face.

For example, if you lose your sense of smell for an unexplained reason, and it lasts longer than 2 to 3 weeks, you should visit your doctor to get it checked out.  

Nasal blockage due to allergies, colds and sinus infections typically affects both sides of the nose. If your symptoms affect only one side of the nose, it could indicate something more serious. This is certainly not always the case, but it¡¯s worth getting checked out.

So, see a doctor if you have symptoms that:

• Are sudden and unexplained
• Affect one side of your face
• Do not go away within 3 to 4 weeks with medicine

How is esthesioneuroblastoma diagnosed?

Your doctor may use several techniques to diagnose esthesioneuroblastoma.

Nasal endoscopy: This is a simple procedure in which your doctor inserts a thin scope with a camera through the nose to examine the inside of your nose. If your doctor finds anything suspicious, you¡¯ll get imaging next.

Imaging tests: Imaging tests tell your doctor the shape, size and location of the mass. A CT scan shows details in the bone. An MRI shows details in the soft tissue. I usually get a CT scan and an MRI because I want to assess the bone and soft tissue.

A PET scan can help your doctor see things an MRI may have missed. CT scans and MRIs are structural tests; they tell us the size, location and shape of the tumor. A PET scan is a functional test. It tells us if there is activity in the tested area. It can also let us know if the cancer has spread.

Biopsy: During a biopsy, your doctor removes a tissue sample to be tested in a lab. A pathologist can then confirm a cancer diagnosis.

How fast does esthesioneuroblastoma grow?

Esthesioneuroblastoma can grow slowly, moderately or quickly. We use the Hyam¡¯s grading system for esthesioneuroblastoma, which categorizes the tumor from Grade I through IV. This tells us how the tumor behaves. Grade I tumors grow the slowest, and Grade IV tumors grow the fastest. In general, we consider Grades I and II tumors to be low grade, while Grades III and IV are more aggressive tumors.

Staging esthesioneuroblastoma

We use the Kadish staging system for esthesioneuroblastoma. Staging tells us if the tumor is growing and how far it has spread.

Stage A: Cancer is confined to the nose.

Stage B: The cancer has spread to the sinuses.

Stage C: The cancer has spread outside of the sinuses to nearby structures, such as the eyes and brain.

Stage D: The cancer has spread to distant parts of the body, such as the lymph nodes in the neck.

How is esthesioneuroblastoma treated?

Treatment for esthesioneuroblastoma will vary based on the size and location of the tumor, as well as your surgical team¡¯s expertise.

Surgery

Surgery is usually the first-line treatment for esthesioneuroblastoma. The goal is to completely remove the tumor. There are two common options for surgery:

• Endoscopic surgery is a minimally invasive approach in which your doctor uses a thin, endoscopic camera and special surgical tools to remove the tumor through the nose.
• Open surgery involves making incisions in the head or part(s) of the face to remove the tumor. The more advanced the cancer is, the more likely you are to need an open approach. Sometimes, surgeons will perform an endoscopic surgery combined with an open surgery to remove a tumor.

At MD Anderson, head and neck surgeons and neurosurgeons work together to perform these surgeries. And because of our experience in treating esthesioneuroblastoma, we can often perform endoscopic surgeries to remove tumors even if they have grown into the skull base or brain.

After we have completely removed the tumor, our second goal is to reconstruct the skull base area so that it is watertight. We don¡¯t want to allow any cerebrospinal fluid to leak into your nose or allow harmful agents to get to your brain. Some patients will also need facial reconstruction.

Radiation therapy

Most patients will receive radiation therapy after surgery to lower the chance of recurrence. Patients with low-grade or very early-stage tumors may not need radiation. We prefer to use proton therapy or intensity modulated radiation therapy (IMRT). The radiation is precise and conforms to the shape of the cancer, which limits damage to nearby structures like the eyes and brain.   

Chemotherapy

Surgery may not be an option for tumors that are too large or if surgery would damage important structures like the eyes. In those cases, the patient may undergo chemotherapy first to shrink the tumor so we can then do surgery.

Treatment to prevent lymph node metastases

We know that esthesioneuroblastoma often spreads to lymph nodes in the neck. But it¡¯s often delayed.

About 10% of patients will have spread to the lymph nodes 5 years after diagnosis; 20% will have spread 7 years after diagnosis and 30% will have spread 10 years after diagnosis.

So, we use radiation to treat the lymph nodes of most esthesioneuroblastoma patients, even if cancer is not present in the lymph nodes at the time of diagnosis. This preventive treatment eliminates the risk of recurrence in the lymph nodes in the neck.

Side effects from esthesioneuroblastoma treatment

A common side effect of esthesioneuroblastoma treatment is loss of sense of smell. Given the tumors¡¯ location, many patients lose some of their sense of smell before starting treatment. And their sense of smell is often completely lost after treatment is over.

If a tumor affects your eyes, treatment may also cause visual problems. We try to correct that with reconstructive surgery, plastic surgery or corrective glasses.   

Endoscopic surgeries and proton therapy have helped us greatly reduce the impact on patients¡¯ appearance and function after treatment. Esthesioneuroblastoma survivors often enjoy a high quality of life.

What is the prognosis for people with esthesioneuroblastoma?

At MD Anderson, we¡¯ve made a lot of advances in how we treat esthesioneuroblastoma. Our overall cure rate is 80 to 85%.

Every patient is unique, so it¡¯s best to talk to your doctor about what you can expect long-term after treatment.

Are there any risk factors for esthesioneuroblastoma?

There aren¡¯t any known risk factors for developing esthesioneuroblastoma.

Esthesioneuroblastoma is diagnosed slightly more often in males. It tends to occur more in two age groups: young adults in their late teens to mid-30s and adults in their 50s and 60s. But this type of cancer occurs in people of any age.

What else should patients and their caregivers know about esthesioneuroblastoma?

Esthesioneuroblastoma sometimes comes back, and that can happen several years after treatment. So, you¡¯ll need lifelong follow-up care. Because esthesioneuroblastoma is such a rare disease, it¡¯s important to seek care at a specialized center like MD Anderson that has experience treating it.

Esthesioneuroblastoma is misdiagnosed at non-specialized centers in about 30% of cases. Treatment is driven by diagnosis, so if the diagnosis is wrong, then you won¡¯t get the right treatment.

When properly diagnosed and treated, we can achieve high cure rates for patients with esthesioneuroblastoma. Expertise is critical in getting the best outcome. And where you go first matters.

, is a head and neck tumor surgeon and medical director of the Head and Neck Center at MD Anderson.

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