Chordoma

Editor-In-Chief: Prab R Tumpati, MD
Obesity, Sleep & Internal medicine
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Chordoma
Chordoma
Synonyms	N/A
Pronounce	N/A
Specialty	N/A
Symptoms	Pain, neurological deficits, headache, visual disturbances
Complications	Metastasis, recurrence
Onset	Typically in adulthood
Duration	Chronic
Types	Conventional chordoma, chondroid chordoma, dedifferentiated chordoma
Causes	Not well understood, possibly genetic mutations
Risks	Genetic predisposition, age
Diagnosis	MRI, CT scan, biopsy
Differential diagnosis	Chondrosarcoma, giant cell tumor, metastatic cancer
Prevention	N/A
Treatment	Surgery, radiation therapy, proton beam therapy
Medication	Targeted therapy, chemotherapy
Prognosis	Variable, depends on location and treatment
Frequency	Rare, approximately 1 in 1,000,000 per year
Deaths	Varies, dependent on treatment success

An MRI image of a patient with a chordoma.

Histopathology of chordoma

Chordoma is a slow growing, very rare kind of cancerous tumor. Chordoma is a type of cancerous tumor known for its slow growth rate and its rarity. Originating from remnants of the notochord, an embryonic structure that contributes to the development of the spine, chordomas primarily manifest within the bones of the spine and at the base of the skull.

Epidemiology

Chordomas are exceedingly rare, making up a small percentage of all primary bone tumors. Their occurrence is observed globally, and no specific geographic, racial, or gender predisposition has been conclusively identified.

Pathophysiology

Chordomas develop from notochordal remnants. The notochord is a vital embryonic structure that plays a pivotal role in the development of the vertebral column. While most of this tissue disappears before birth, small remnants may persist in certain parts of the spine or base of the skull. It is from these remnants that chordomas can arise.

Clinical Presentation

Due to their slow-growing nature, chordomas may not produce symptoms until they reach a significant size. When symptoms do emerge, they are typically due to the tumor exerting pressure on adjacent structures. For instance:

• Tumors in the spine can lead to pain, weakness, or numbness in the limbs.
• Tumors at the base of the skull can affect vision, hearing, and other cranial functions.

Diagnosis and Treatment

• Diagnosis: Imaging tests such as MRI or CT scans are often employed to identify the presence of the tumor. A biopsy, where a small sample of the tumor is extracted and examined under a microscope, is definitive for diagnosis.
• Treatment: Management of chordoma typically involves surgery to remove as much of the tumor as possible. Given the tumor's location, complete surgical removal can be challenging. Radiation therapy may be employed post-surgery to target residual tumor cells.

Prognosis

Although chordomas grow slowly, they can be recurrent and may spread to other parts of the body. Regular monitoring and follow-ups are crucial for patients diagnosed with chordoma.

See Also

• Cancer
• Tumor
• Bone tumor
• Spinal cord

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