What is Chordoma & How is it Treated?

Chordoma is a pathological condition in which there is development of malignant tumors in the bones, especially of the spine and the base of the skull. Chordoma in itself is an extremely rare condition of all malignant condition involving the bones roughly amounting to less 1% cases.

The location of Chordoma makes it a difficult condition to treat due to its close proximity to extremely vital structures of the body like brain and spinal cord even though these tumors are relatively slow growing.

The mainstay of treatment for Chordoma is a combination of surgery with radiation and chemotherapy. The reason as to how Chordoma develops is that, there is a cartilage like structure in the fetus when the baby is developing which facilitates formation of the spine. Once the spine is formed it replaces the notchcord. This happens during the six months of fetal development. In some cases, some part of the notchcord remains and this is where Chordomas originate from.

Chordoma tends to occur mostly in the sacrum, coccyx, or at the base of the skull, although they may occur anywhere along the length of the spine. The usual age of onset of Chordoma is between 50 and 70 years of age, although in some rare cases young individuals and even children may have Chordoma.

Chordoma is quite a serious and life threatening condition and tends to spread to other areas of the body, usually the lungs and thus early detection and treatment is of extreme importance.

As stated above, the root cause of development of Chordoma is the remains of notchcord that is present during fetal development and facilitates development of the spine.
This notchcord is replaced by the spine during the six weeks of development of the fetus but in some cases some parts of the notchcord still remains and this later results in development of Chordoma.

What really causes these cells of the notchcord to become malignant is still not very clear and research is going on to identify this one aspect of the formation of Chordoma.

Since Chordoma is a slow growing tumor, the patient may be having symptoms for many years before he or she looks for a treatment of the symptoms. The symptoms of Chordoma depend on the location of the tumor and include:

If the tumor is at the base of the skull then the patient may have symptpms of frequent headaches or vision disturbance

If the tumor is present in the spine then the patient may experience symptoms of pain in the area of the tumor namely the neck, back, or sacrococcygeal area. If the tumor compresses an adjoining nerve then the symptoms experienced by the patient may mimic that of a herniated disc and the symptoms may include pain, weakness, numbness and tingling in the upper and lower extremities.

If the tumor is present in the groin area then the patient may experience symptoms of groin pain, bowel and bladder dysfunction, numbness and tingling in the lower extremities, and weakness of the lower extremities.

In order to diagnose Chordoma the physician will take a detailed history of the patient as to when the symptoms started. A thorough physical examination will be performed to inspect the area of pain and weakness.

Advanced radiological studies in the form of CT and MRI scan of the affected area may be done which will clearly indicate the presence of a tumor. Once a tumor is identified then a biopsy of the tumor is taken which in cases of Chordoma will be malignant and thus will confirm a diagnosis of Chordoma. Once Chordoma has been diagnosed the next step is to stage the tumor which is helpful in formulating a treatment plan for the patient.

For this, other investigational studies will be performed to see if the tumor has spread to other parts of the body and will include blood tests, a CT scan of the lungs, and a bone scan to look at the status of the bones which will get affected due to the Chordoma.

As stated above, Chordoma is very difficult to treat due to its close proximity to the vital structures of the body like the brain and spinal cord. Chemotherapy and standard radiation usually does not cure the problem.

Thus, surgery is most often recommended as the best treatment plan for treating Chordoma. This is combined with radiation treatments. If the patient is a poor candidate for surgery due to medical comorbidities or if the tumor is located in a place where surgical excision is not possible, then radiation treatment is done alone to help slow down the progress of the tumor.

Proton beam therapy is a very novel and effective way of treating and slowing down the progression of Chordoma. The best way to perform surgical removal of the tumor is to remove the tumor along with some healthy tissue along with it.

This may result in some neurological dysfunction like when a tumor is removed from the sacrum then it may lead to some bowel and bladder dysfunction or lower extremity weakness but on the brighter side it will remove the tumor and prevent spreading of Chordoma.

The overall prognosis of Chordoma is dependent on various factors. It depends on the location of the tumor, the treatments that can be given, the response of the patient to those treatments, age and overall health of the patient and the like. A skilled surgeon is the best person to suggest a prognosis for a condition like Chordoma but with early detection and appropriate treatment many individuals live a normal life and get cured from Chordoma.