If 2 members of a family have chordoma, it is recommended that other family members are investigated over a period of time to see if they are developing chordoma. There are a handful of families around the world in which a number of family members have developed chordoma: that is, they have a ‘familial form’ of the disease.
The reason why a person develops chordoma is unknown other than the link to notochord persistence. The majority of those who develop chordoma do not have a family history of the disease that is, it occurs sporadically. Chordomas at the base of the skull occur more commonly in younger people. It is slightly more common in men than women (1.6:1), and is even less common in the black African population. It is estimated that 500 people in the UK are currently living with the disease.
It occurs in approximately 1 in 800,000 people (incidence) and the number of people currently living with chordoma is approximately 8 per million of the population (prevalence). Chordoma does not discriminate on age, sex, ethnicity, geography or sexual orientation. More about Chordoma symptomsĬhordoma can occur in any age group, from infancy to the very elderly although it is more commonly found in individuals over the age of 40 and most typically in those in their mid-50s. Furthermore, as the symptoms can mimic other common problems, such as back ache, and given chordoma is so rare, it is often takes a long time for a definitive diagnosis to be made, as most GPs, general physicians and surgeons during their career will never meet a patient with chordoma. Chordoma is slow-growing and, those tumours arising in the sacrum and lower back, in particular are often significant by the time they are diagnosed. If it occurs in the vertebral bodies or the bones of the sacrum or coccyx (tail bone), it generally causes persistent back ache and may cause numbness or weakness of the arms and legs and impact on bowel or bladder function. Occasionally it may affect facial sensation or movement, speech and throat functions. At the base of the skull, it can cause headaches, neck pain, and blurred or double vision. The most common signs however are variable pain and neurological changes. People experience different symptoms depending on where their tumour arises. Chordoma diagnosis and treatment What are the signs and symptoms of chordoma?