Chordoma is a rare type of cancerous tumor that originates from remnants of the notochord, an embryonic structure present during early development. The notochord is a rod-like structure that plays a crucial role in the formation of the spinal column and the base of the skull. Normally, the notochord disappears as the spine develops, but small remnants can persist into adulthood. These leftover cells can sometimes give rise to chordomas, which are slow-growing tumors that typically occur along the spine, from the base of the skull down to the tailbone.
The exact cause of chordoma is not fully understood, but it is believed to arise from these persistent notochordal cells that undergo abnormal changes. These changes cause the cells to multiply uncontrollably, forming a tumor. Unlike many other cancers, chordomas do not usually spread quickly but can invade nearby bones and soft tissues, making them difficult to treat. They often recur after treatment and can metastasize, or spread, to other parts of the body such as the lungs in some cases.
Genetic factors may play a role in chordoma development. Research has identified that the tumor cells often express a protein called brachyury, which is involved in notochord development and is considered a key marker for chordoma. This protein is encoded by the TBXT gene, and abnormalities in this gene may contribute to the formation of chordomas. However, chordomas are not typically linked to inherited genetic mutations, meaning they usually occur sporadically without a clear family history.
Environmental factors or lifestyle choices have not been clearly associated with chordoma risk. Because chordomas arise from embryonic tissue remnants, their development is more related to cellular and molecular changes rather than external causes. The rarity of chordoma also means that large-scale studies to identify specific causes are limited.
In summary, chordoma is caused by the abnormal growth of residual notochord cells left over from early development. These cells undergo genetic and molecular changes that lead to tumor formation along the spine or skull base. While the precise triggers for these changes remain unclear, the involvement of the brachyury protein and TBXT gene is a significant focus of ongoing research. The tumor’s slow growth and tendency to recur make it a challenging condition to manage clinically.
