Dear OncoLink "Ask the Experts,"
I am trying to find information regarding esthesioneuroblastoma. What is it? How rare is it? What kind of treatment is recommended? What is the prognosis for one who has it?
John Han-Chih Chang, MD and Kenneth Blank, MD, Editorial Assistants for Oncolink, respond:
Thank you for your question.
Esthesioneuroblastoma is a very rare tumor, which occurs in the nasal cavity. Between 1978 and 1990 only 84 esthesioneuroblastomas were reported in the United States. They are believed to arise in the olfactory (smelling) nerves that originate in the nose and travel into the brain. The tumors have a bimodal age distribution occurring most commonly in teenagers and the sixth decade of life.
Most patients with esthesioneuroblastoma present with symptoms of nasal obstruction (inability to breath through the involved nostril), nosebleed or both. These tumors can spread to the lymph nodes and to other areas of the body. The incidence of metastasis (spread to other body areas) is ~50%.
Treatment depends on tumor stage. Early stage tumors (those which are small and confined to the nasal cavity) can be successfully treated with surgery, radiotherapy or both. Tumors which have extended beyond the nasal cavity have a high risk of distant spread and chemotherapy is often recommended in addition to surgery and radiotherapy.
Prognosis also depends on the tumor stage. Early tumors are often well controlled with either surgery or radiotherapy. However, because of the rarity of these tumors it is difficult to determine the true prognosis.