Chordoma Treatment in Mumbai, India

Overview

Chordoma is a type of cancer that forms tumors in parts of the spinal cord and the base of the skull. These tumors develop primarily in the anterior part of the spine or the base of the skull and can run down to any part of the spinal cord.

This is a rare disease and the incidence is approximately 1 per million people per year. Since it occurs in very few people, it is difficult to determine its prognosis. According to research, people diagnosed with chordoma survive for at least 7-10 years post-diagnosis and treatment. Even without any treatment, this condition is life-threatening and the natural progression is about 5-7 years.

Chordoma is a rare congenital disorder for which pathophysiology has not been studied extensively. However, it is known that genetic mutations occur in the DNA sequence that give rise to a deformed protein called brachyury, which causes abnormal cell division causing tumor growth.

Signs and Symptoms

Chordoma is a slow-growing tumor, often the patients are symptoms free till a certain age. Since the tumor is located near the nerves, most of the symptoms are neurological, such as:

Headaches- cluster headaches
Pain- arms, back, and legs
Visual Problems- double vision, nerve or muscle weakness
Paralysis of the facial nerve
Fatigue and tiredness

As the tumor can grow anywhere near the skull or the spine, the signs and symptoms vary significantly. These signs and symptoms are quite common and can occur in other conditions as well, which makes it tricky to detect the tumor.

Symptoms of tumor at the base of the spine:

Lower back pain
Tenderness and inflammation
Lump in the back
Constipation and loss of bladder control

Symptoms of tumors in the neck:

Pain and inflammation
Breathing obstruction
Dysphagia (difficulty swallowing)

Causes of Chordoma

Chordoma develops when the collection of cells called notochord that form the discs of the spinal cord in the early stages of development are not completely utilized. Some of these cells then form into tumors that are malignant and spread throughout the spinal cord. Most of the cells are gone by the time a child grows, but some may become cancerous. Genetic mutations that cause the production of protein is a major cause for developing this tumor.

Types of Chordoma

Chordoma is differentiated based on the type of tumor, its location, size, and pathology:

Conventional chordoma: It is a classic case of tumor that accounts for about 70-80% of the most common chordoma.

Dedifferentiated chordoma: This type of tumor is aggressive and accounts for less than 5% of all the tumors. It typically is found near the base of the spine but can occur anywhere on the spinal cord or the skull.

Chondroid tumors: These types of tumors appear at the base of the spine and account for almost 10-25% of all the tumors. They usually arise and grow due to abnormal cartilage tissues and the abnormal notochord. It’s characteristics are similar to chondrosarcoma, a type of bone cancer.

Risk Factors

There are no known environmental risk factors of chordoma. The development of tumors do not have any known causes but is mostly attributed to genetics. It is known that more than 95% of patients with chordoma have a DNA mutation that causes them to produce a protein called Brachyury, a major risk factor for developing this condition. It is a very rare condition and 1 in a million people are affected by this.

Diagnosis of Chordoma

Chordoma is diagnosed using two methods- computerized tomography and biopsy.

A detailed image of the skull and the spinal cord to locate the tumor and its size.
Biopsy tests examine a sliced piece of the tumor to determine if they are cancerous in nature.

Possible Treatment of Chordoma

Possible treatments include surgery, stereotactic radiosurgery, and targeted drug therapy. These treatments depend on the location and size of the tumor.

Surgery: The advantage of surgery is the effective removal of a tumor. However, if it is present in near-critical tissues, the removal might not be successful. This might lead to the use of alternative methods of removal.
Stereotactic Radiosurgery: Powerful and precise radiation is used to target the affected areas when surgery is not an option. This may be effective in removing the cancer cells but also has the disadvantage of affecting the surrounding tissues.
Targeted Drug Therapy: Specialized drugs that have an affinity towards cancer cells and possess a differentiating ability between cancer and normal tissue are utilized in this mode of treatment. Unlike surgery, the treatment has the potential to eradicate most, if not all, cancer cells. A follow up of such treatment with cancer marker detection allows the physician to track the effectiveness of the treatment. Such precise and controlled treatment can be used to give a patient a calibrated therapy.
Proton Beam Therapy (PBT): PBT is a kind of radiotherapy that utilizes light emissions (invigorated particles), rather than light emissions beams (photons) that are utilized in ordinary radiotherapy. It is more effective than regular radiotherapy, thus causing less harm to the sound tissue encompassing cancer and different organs. It can also be used for a few malignant bone growths where the disease is near a basic piece of the body like the spinal line.

One of the key advantages of PBT is that it is more focused, hence reducing the damage to healthy tissue. This means fewer side effects and more benefits of the therapy.

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