Chordoma can be defined as a rare type of bone cancer or sarcoma that primarily arises in the bones of the spine or the skull. In most cases, it forms where the skull sits atop the spine (skull base) or at the bottom of the spine (sacrum) or at the vertebrae of the mobile spine. Chordoma mainly initiates in the cells of the notochord (a structure that’s present in the developing embryo and is important for the future development of its spinal disks). Most of these cells wither away or perish by the time a child is born or soon after. But sometimes a few of these cells remain behind embedded in the bones of the spine or at the base of the skull and, rarely turn into malignant tumours.

Although Chordoma can occur at any age, it is commonly noticed in adults between ages 40 and 60. Chordomas are typically slow-growing malignant tumours, but they are hard to treat as the tumours are usually located near the nervous system tissue. They also have a higher chance of recurring in the original location even after complete treatment. Sometimes, Chordomas metastasize to surrounding healthy tissues like; other bones, lymph nodes, liver, skin, and lungs.

Also Read: Chondrosarcoma: Causes, Symptoms And Treatment

Types Of Chordomas

According to WHO, there are three main types of Chordomas:

  • Classic/conventional chordoma (makes up 80% to 90% of all chordoma cases)
  • Dedifferentiated chordoma (records less than 5% of cases)
  • Poorly differentiated chordoma (fewer than 60 cases detected in medical history)

Causes

Although the exact cause of Chordoma is yet unknown some studies correlate it to changes in the TBXT gene. Some families who have had multiple members suffering from chordoma were found to inherit a duplication of the TBXT gene. Mutations in this TBXT gene triggered abnormal growth of remnant notochord cells within the spinal cord. These cells grew both in size and number and kept piling up without perishing to form malignant tumourous structures.

Also Read: Osteosarcoma: Causes, Symptoms And Treatment

Risk factors

Certain causative factors that increase the risk of Chordomas include:

Age: Adults falling in between the ages 40 - 60 are more at risk of Chordoma

Gender: Women are at a higher chance of getting Chordoma than men

Genetic condition: People with a hereditary condition called Tuberous sclerosis are at a higher incidence of developing chordoma.

Exposure To Radiation: Undergoing any radiation treatment in the past owing to some cancerous condition or getting exposed to radiation daily can make a person more prone to Chordoma

Symptoms

The characteristic symptoms of Chordoma are difficult to acknowledge as the tumour is slow-growing and the symptoms usually crop up late. Since the tumour grows near the brain or spinal cord, the pressure ensuing from it causes the characteristic manifestations. These include:

Chordoma in the skull:

  • Pain or weakness in the muscles of your back, arms or legs
  • Facial pain or numbness
  • Double vision
  • Blurry vision
  • Intense headaches

Chordoma in the tailbone:

  • A growing lump that you can feel through the skin's surface
  • Problems with bladder or bowel function
  • Lower back pain or tailbone pain

Diagnosis And Treatment

If you recognize any of the above-mentioned signs or symptoms, do talk to a doctor right away to get diagnosed and treated at the earliest. The doctor usually does a thorough physical check-up to detect any lumps or masses on the bones, slightly presses the area near the lower back to understand if it emits pain, discerns the patient’s own and family medical history and conducts some diagnostics. These include:

  • Neurological examination
  • Imaging procedures comprising bone scan, CT scan, MRI scan, X-ray, and Ultrasound
  • Needle Biopsy

Grading

To treat cancer effectively, the oncologist usually grades the tumour to locate its extent of spread to fight it better:

Low grade (I): When the tumour is slow-growing and is limited to the place of origin

Intermediate grade (II): This tumour grows and spreads more quickly than the Grade I type and might spread to the nearby structures and lymph nodes

High grade (III): These are the fastest growing ones and can often spread to other healthy tissues and distant organs

Treatment

Once the tumour is graded, the group of doctors usually settles upon the best possible treatment to give a healthy life to the patient. The available treatment options include:

  • Surgery
  • Radiation therapy
  • Chemotherapy
  • Immunotherapy
  • Targeted therapy
  • Clinical trials
  • Palliative care