Cranial Conditions

Chordomas Tumors

Chordomas Tumors

Chordomas tumors are a rare but malignant tumor that can take place anywhere along the spine.

What are Chordoma Tumors?

Chordomas represent a rare form of malignant tumors, which can manifest anywhere along the spinal column. When occurring at the base of the skull, they are specifically termed skull base chordomas. Typically characterized by a slow growth rate, these tumors possess the potential to infiltrate surrounding tissues and organs. The classification of chordomas hinges on their aggressiveness in terms of local spread, with three distinct subtypes identified:

  • Chondroid chordoma: The least aggressive variant among chordomas.
  • Conventional or Classic chordoma: Exhibiting a higher degree of aggressiveness compared to the chondroid type.
  • Dedifferentiated chordoma: Identified as the most aggressive and rapidly proliferating subtype of chordomas.

Of these, conventional or classic chordomas emerge as the most prevalent subtype found within the skull base.

What Causes Chordoma Tumors?

The precise etiology of chordomas remains elusive. Generally, tumors arise from abnormal cellular proliferation devoid of physiological purpose. Cell division is typically regulated by tumor suppressor genes, which also play a role in DNA repair. These genes serve as a counterbalance to oncogenes, which promote cancer development. Mutations affecting protein encoding within tumor suppressor genes can lead to dysregulated cell division and subsequent tumor formation.

Ideally, the body’s immune system should identify and eliminate aberrant cells. However, tumors may evade immune detection through the secretion of substances obstructing recognition, allowing unchecked growth.

Symptoms and Diagnosis

Due to their slow growth, chordomas may initially evade detection as patients may not exhibit symptoms. Once symptoms manifest, they vary depending on the tumor’s location. Skull base chordomas may present with headaches and vision disturbances, while spinal chordomas can induce localized pain, particularly if nerves are compressed. Tumors situated in the coccyx region may result in numbness and bowel or bladder dysfunction. Diagnosis typically involves a combination of clinical observation and imaging tests such as x-rays, magnetic resonance imaging (MRI), and computed tomography (CT) scans.

How are Chordoma Tumors Treated?

Given the proximity of chordomas to critical structures like the brain and spinal cord, treatment poses significant challenges. Surgery often emerges as the primary therapeutic modality, leveraging advanced techniques and technology. Additionally, certain radiation therapies may offer efficacy in managing chordomas.

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