Which are the causes of Chordoma?

Chordoma is a rare type of cancer that originates from the remnants of the notochord, a structure that forms during early embryonic development and eventually disappears. While the exact cause of chordoma is not fully understood, several factors have been identified that may contribute to its development.

Genetic Factors: Research suggests that genetic mutations play a significant role in the development of chordoma. In particular, a gene called T gene (also known as brachyury gene) has been found to be associated with chordoma. Mutations in this gene can disrupt normal cell growth and division, leading to the formation of tumors. However, it is important to note that not all individuals with chordoma have identifiable genetic mutations, indicating that other factors may also be involved.

Environmental Factors: Exposure to certain environmental factors may increase the risk of developing chordoma. However, these factors have not been extensively studied, and their specific role in chordoma development remains unclear. Some studies have suggested a potential link between chordoma and exposure to ionizing radiation, such as radiation therapy for other cancers. Additionally, there have been rare cases where chordoma has developed at the site of previous surgical procedures or trauma, suggesting a possible association with tissue damage.

Age and Gender: Chordoma typically occurs in adults, with the average age of diagnosis being around 50 years. It is more commonly diagnosed in males than females. The reasons for these age and gender differences are not well understood, but they may be influenced by hormonal factors or differences in genetic susceptibility.

Hereditary Conditions: In some cases, chordoma can be associated with certain hereditary conditions. For example, individuals with a rare genetic disorder called Tuberous Sclerosis Complex (TSC) have an increased risk of developing chordoma. TSC is characterized by the growth of noncancerous tumors in various organs, including the brain and kidneys. Chordoma can also occur in individuals with a condition called hereditary chordoma susceptibility syndrome, which is characterized by an increased risk of developing chordoma and other related tumors.

Conclusion: While the exact causes of chordoma are not fully understood, it is believed to result from a combination of genetic and environmental factors. Genetic mutations, particularly in the T gene, are thought to play a significant role in chordoma development. Environmental factors, such as exposure to ionizing radiation, may also contribute to the risk. Age, gender, and certain hereditary conditions can further influence the likelihood of developing chordoma. Further research is needed to gain a deeper understanding of the causes of chordoma and to develop more effective prevention and treatment strategies.