Esthesioneuroblastoma

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.