Chordoma


Definition

Chordoma is an uncommon cancer that mostly occurs in the spine and bones of the skull. The condition can also be categorized as a type of sarcoma which is a term that is generally used for a variety of cancers that affects the connective tissue and bone.

Chordomas are malignant tumors that grow moderately slow and metastasize to other organs of the body. The tumor arises from the notochord during the early stages of fetal development. It is more observable among individuals who are 55 years old and above, although younger people can be affected. Chordomas are also life-threating and could cause death by metastasizing to other organs.

Symptoms of Chordoma

Symptoms of chordomas depend on its location and size. The tumors usually develop at the clivus which is situated in front of the brainstem, sacrum which is the triangular bone close to the bottom of the spine, and cocyx which is at the bottom part of the vertebral column.

Symptoms that may possibly occur are:

  • Double or blurred vision
  • Headache
  • Neck pain
  • Changes in facial movements, speech, and voice
  • Swallowing problems
  • Changes in bladder or bowel function
  • pain, tingling, and weakness of the legs and arms
  • Noticeable lump
  • Numbness in the crotch region

Types

Chordomas are divided into three:


Chondroid chordomas

Comprises both chondromatous and chordomatous features and are more common in individuals younger than 40.

Conventional chordomas

It is the most common type of chordomas and is distinguished by the nonexistence of mesenchymal or cartilaginous components.


Dedifferentiated chordomas

These are tumors that can grow rapidly and spread to other body parts.

Causes & Pathology

The primary causes of chordoma are not known. Most tumors suddenly arise and are not caused by any inherited genetic change. An existing theory which states that acquired mutations or abnormalities lead to malignant growth of notochordal remnants.

A case where a person genetically inherits the tumor is very rare. Gelatinous and fluid mucoid material is noted within the tumor. The accumulation of calcium and sequestered bone fragments may also be found in some individuals.

Tumors that spread to other areas of the body are detected in about 7-14% of patients. Chordomas are sporadic which means that they arise as the product of mutations that randomly occurred in a person’s life. There are no specific environmental factors that are linked to chordoma, although it can somehow contribute to its sporadic mutations. More research is needed in order to point out the exact cause of the condition.

Diagnosis

Symptoms of chordomas are not specific that is why the diagnosis is based upon the features of radiologic pathologic findings. Imaging tests can help the surgeons in detecting the presence of a tumor, its location, size, and local extension. Through these tests, surgeons can plan out certain surgical procedures that might be indicated for treatment. Tests may include:

X-Rays

The damaged bones can be seen on the x-rays although the x-ray images of the sacrum are frequently hard for physicians to interpret.

Computed tomography (CT) scans

It is a test wherein a computer makes use of x-rays to produce cross-sectional body images.

Magnetic resonance imaging (MRI) scans

It is a test that uses radio waves and magnetic fields to create cross-sectional images of the affected area.


After any of the tests is done, an incision biopsy wherein a needle is inserted through the skin to get small sample of cells is required to confirm a diagnosis of the chordoma tumor. Some cases even necessitate a surgical procedure to obtain enough tissue for diagnosis. The tissue will be examined under a microscope to specify the type of tumor present.

Treatment

Treating chordomas is really difficult mainly because the tumors are close to the spinal cord and brain. Chemotherapy has not been quite potent, and radiation alone can hardly cure the tumor.

If possible for the patient’s overall health status, surgery may be recommended. Surgery combined with radiation is frequently used in treating chordomas. If the tumor cannot be removed through surgery, radiation therapy may sometimes stop the growth of the tumor.

Although surgery seems to be the best treatment option, it is very difficult to perform in several cases due to the structures close to the tumor. Some operations result to a loss of neurologic function.

Foundation

The Chordoma foundation is a nonprofit or non-business entity that works to enhance the lives of patients with the condition by speeding up their research in order to come up with effective treatments for chordoma, and also by helping patients to have the best care possible.

References

  1. Tumor Location | Types of Chordoma | Incidence and Prevalence | Demographics | Risk Factors Signs and Symptoms | Treatment | Prognosis and Survival | Further Reading at http://www.chordomafoundation.org/understanding-chordoma/
  2. Chordoma Treatment at http://orthoinfo.aaos.org/topic.cfm?topic=A00084
  3. https://rarediseases.org/rare-diseases/chordoma/
  4. Mima M, Demizu Y, Jin D, et al (2014 Jan). Particle therapy using carbon ions or protons as a definitive therapy for patients with primary sacral chordoma. Br J Radiol. 87(1033):20130512.
  5. Xia M, Huang R, Sakamuru S, Alcorta D, Cho MH, Lee DH, et al (2013 May 10). Identification of repurposed small molecule drugs for chordoma therapy. Cancer Biol Ther. 14(7)
  6. Amer HZ, Hameed M (2010/02) . Intraosseus benign notochordal cell tumor. Arch Pathol Lab Med. 134:283-288.

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