Overview

Esthesioneuroblastoma, also known as olfactory neuroblastoma, is a malignant tumor that develops in the upper nasal cavity. It usually originates in the nasal cavity and can spread to the sinuses, eyes, and brain. Esthesioneuroblastoma is a rare form of cancer. It can severely affect one’s sense of smell and breathing.

Esthesioneuroblastoma starts in the area near the bone that separates the nasal cavity from the brain. The bone has very small holes through which the olfactory nerves travel. Esthesioneuroblastoma can spread to the lymph nodes in the neck and the parotid glands. It also can spread to other areas of the brain and the body in severe cases, including the lungs, liver, and bones.

People with esthesioneuroblastoma may lose their sense of smell, have frequent nosebleeds, and have trouble breathing through their nostrils. Common treatments for this condition usually include surgery, radiation, and chemotherapy.

Symptoms

The symptoms of esthesioneuroblastoma may change as the condition progresses. Common symptoms include:

  • Decreased or loss of sense of smell
  • Recurrent nosebleeds
  • Trouble breathing via the nose
  • Pain in the area surrounding the eyes
  • Vision loss or alteration
  • Pain or pressure in the ear
  • Frequent headaches
  • Nasal obstruction
  • Congestion or stuffiness that worsens or does not improve
  • Pus in the nose
  • Neck lymph nodes enlargement
  • Having difficulty opening the mouth

If any of the signs and symptoms of esthesioneuroblastoma persists, consult a doctor right away for proper diagnosis and treatment.

Causes

The cause of esthesioneuroblastoma is currently unknown. Cancer, in general, originates with a genetic mutation that causes normal, healthy cells to continue growing despite signals to halt, which normal cells do not do. Cancer cells proliferate and reproduce uncontrollably. The aberrant cells that accumulate produce a tumor.

Risk factors

Research suggests that individuals who are exposed to hazardous chemicals and particles either through their occupation or other means may have a higher risk of developing nasal cavity cancers. Substances such as wood dust, wheat, nickel and cadmium dust, glues, formaldehyde, and solvents are among the potential culprits. While the role of tobacco smoke in the development of olfactory neuroblastoma is uncertain, it may be a contributing factor.

Diagnosis

To diagnose esthesioneuroblastoma, the medical professional typically begins by discussing the patient’s symptoms, reviewing their family’s medical history, and conducting a physical examination. The doctor will likely examine the eyes, nose, head, and neck to assess the tumor’s size and spread.

The following tests may be required to confirm the diagnosis:

  • Endoscopy: This procedure allows the doctor to examine the extent of the tumor in the nose, back of the nose or nasopharynx, and sinus area of the nasal cavity. This procedure uses an endoscope, a small, flexible tube connected to a camera to see the inside of the nose.
  • Imaging tests: Imaging tests can help assess the location and extent of the esthesioneuroblastoma, and whether it has spread. To examine the nasal cavity, a variety of imaging techniques may be used such as X-rays, CT scans, MRI, and positron emission tomography (PET).
  • Biopsy: Esthesioneuroblastoma is difficult to diagnose because it is extremely rare and might resemble other malignancies that arise in the head, neck, or nasal areas. A biopsy may be necessary to obtain more information. This is a surgical technique that involves removing a sample of tumor tissue for pathological investigation.

As part of the diagnostic process, the doctor may insert specialized equipment through the patient’s nose to obtain tissue samples from the tumor. Accurate testing and analysis of the biopsy sample are necessary to distinguish esthesioneuroblastoma from other tumors that may look similar. The biopsy results will also help determine the tumor’s grade, or level of aggressiveness, through pathological investigation. Typically, a biopsy is conducted in a doctor’s office or clinic.

Treatment

Esthesioneuroblastoma treatment mainly entails removing the malignancy through surgery. After surgery, a mix of radiation and chemotherapy treatments can offer the best chance of survival. Experts from different fields, such as neurosurgeons, head and neck surgeons, radiation oncologists, and medical oncologists, are frequently involved in the treatment of esthesioneuroblastoma.

  • Surgery: Depending in the location of the tumor, common surgical procedures to treat esthesioneuroblastoma include:
    • Endoscopic surgery. Special surgical tools are passed through the endoscope to help with the removal of the malignancy and surrounding tissue. An endoscope is a long, thin tube with a camera inserted into the nose.
    • Craniotomy. In this skull base surgery, the tumor is removed and separated from the brain. A tiny part of the skull is temporarily lifted to gain access to the tumor.
  • Radiation therapy: Radiation therapy is commonly used to treat esthesioneuroblastoma. This could be the primary treatment for people not eligible for surgery, or it could be administered after surgery to lessen the likelihood of the cancer returning. To kill cancer cells, radiation therapy employs high-energy beams such as X-rays or protons. After surgery, people with esthesioneuroblastoma frequently receive radiation therapy to eradicate any microscopic cancer cells that may linger in the head and neck.
  • Chemotherapy: Chemotherapy may also be used to treat esthesioneuroblastoma. Chemotherapy is used with radiation therapy following surgery to eliminate any cancer cells that may persist, especially in tumors that are very aggressive or widespread. Chemotherapy uses strong medications to kill cancer cells.

Doctors who treat this condition