Esthesioneuroblastoma
Encyclopedia
Esthesioneuroblastoma is a rare form of cancer
involving nasal cavity
and believed to arise from the olfactory epithelium. It can cause loss of vision, sight and taste.
It is often considered synonymous with "olfactory neuroblastoma
", but the tissue of origin is not yet well characterized. Associated with Trisomy 8
.
It was first characterized in 1924.
, only 200 cases of the disease have been recorded worldwide in the past two decades.
A 1997 literature search identified 1,457 cases in the published literature since its discovery in 1924, however 487 were cited in more than one paper, bringing the total of reported cases to 945.
, who was suffering from the disease and ended her life after being denied euthanasia.
Cancer
Cancer , known medically as a malignant neoplasm, is a large group of different diseases, all involving unregulated cell growth. In cancer, cells divide and grow uncontrollably, forming malignant tumors, and invade nearby parts of the body. The cancer may also spread to more distant parts of the...
involving nasal cavity
Nasal cavity
The nasal cavity is a large air filled space above and behind the nose in the middle of the face.- Function :The nasal cavity conditions the air to be received by the other areas of the respiratory tract...
and believed to arise from the olfactory epithelium. It can cause loss of vision, sight and taste.
It is often considered synonymous with "olfactory neuroblastoma
Neuroblastoma
Neuroblastoma is the most common extracranial solid cancer in childhood and the most common cancer in infancy, with an annual incidence of about 650 cases per year in the US , and 100 cases per year in the UK . Close to 50 percent of neuroblastoma cases occur in children younger than two years old...
", but the tissue of origin is not yet well characterized. Associated with Trisomy 8
Trisomy 8
Trisomy 8 is a human chromosomal disorder caused by having three copies of chromosome 8. It can appear with or without mosaicism.-Characteristics:...
.
It was first characterized in 1924.
Incidence/prevalence
According to the BBCBBC
The British Broadcasting Corporation is a British public service broadcaster. Its headquarters is at Broadcasting House in the City of Westminster, London. It is the largest broadcaster in the world, with about 23,000 staff...
, only 200 cases of the disease have been recorded worldwide in the past two decades.
A 1997 literature search identified 1,457 cases in the published literature since its discovery in 1924, however 487 were cited in more than one paper, bringing the total of reported cases to 945.
Treatment
Several surgical approaches have been described, but post-excision recurrence rates have remained relatively high.Notable cases
The disease was brought into prominence by the case of Chantal SébireChantal Sébire
Chantal Sébire was a retired French teacher who suffered from esthesioneuroblastoma, a rare form of cancer, and fought for the right to die through euthanasia.-Background:...
, who was suffering from the disease and ended her life after being denied euthanasia.