Chordoma is a rare, slow growing malignant tumor that develops in the spine and skull bones. It is thought to form from the remnants of the notochord (a preliminary structure present in a developing baby in the womb, which eventually forms the spinal cord). The exact cause of a chordoma has not been established with certainty.
Chordomas are more common in adults than children. They can be life-threatening and difficult to treat and therefore require specialized follow-up care.
The most evident signs of a chordoma are pain and neurological changes. Symptoms typical to a skull-based chordoma include headache, issues with vision such as double vision, and pain in the neck region. Symptoms vary depending on the size of the tumor. For instance, a large chordoma may bring about changes in voice and speech, as well as changes in facial sensation or movement and swallowing function. Some other symptoms include weakness in the limbs, pain, body pain, and changes in the functioning of the bowel or the bladder.
A sarcoma is a rare cancerous tumor or mass of abnormal tissue originating from either the soft tissues or the bones. Tumors may be benign or malignant. Benign tumors are not cancerous and do not spread to other parts of the body whereas. Sarcomas are malignant tumors which are cancerous and can spread to other parts of the body.
A soft tissue sarcoma is a type of cancer that originates in the soft tissues of the body including fat, muscle, nerves, fibrous tissue, blood vessels, or deep skin tissues. This type of sarcoma can appear on any part of the body, but most commonly manifests in the extremities (arms or legs). Soft tissue sarcomas can also occur in the trunk, head and neck area, internal organs, or the retroperitoneum, the back of the abdominal cavity.
Bone sarcomas are cancerous tumors that develop in bones. Bone sarcomas may grow, compressing and destroying the healthy bone tissue.
Bone sarcomas are rare but the most common types include:
- Ewing’s sarcoma
Osteosarcoma is a common type of bone cancer that usually starts in the long bones of the arms and legs. It occurs in teenagers during the period of rapid growth and is more common in boys than girls. Osteosarcoma normally develops within the cells that form the bone tissue (osteoblasts).
Children with retinoblastoma (cancer of the eye) are at greater risk of developing osteosarcoma. Teenagers who are taller than average are predisposed to developing osteosarcoma. Children exposed to prior radiation treatment for cancer are also more likely to develop osteosarcoma. Adults may be at a higher risk if they have Paget’s disease or previous exposure to high doses of radiation therapy.
Chondrosarcoma is a malignant tumor that develops in the cartilage or the connective tissue covering the ends of the bones. Chondrosarcomas can occur both on and within a bone and most frequently affect the pelvic bones, shoulder bones, and the upper parts of the arms and legs. Most of these tumors are slow growing and can occur at any age. The incidence is found to be higher in males than females.
The cause of chondrosarcoma is unknown, but hereditary conditions such as Ollier disease, Maffucci syndrome, and hereditary multiple exostoses (HME) can enhance the risk of chondrosarcoma occurrence. People who have had chondroma (benign bone tumor) or an osteochondroma run the risk of developing chondrosarcoma.
Ewing’s sarcoma is a malignant tumor that normally starts in the bone. It commonly affects teenagers and young adults who are transitioning through puberty. Ewing’s sarcoma starts in the bone, but it can also affect the soft tissues. This is known as extraosseous Ewing’s sarcoma. It can develop in any bone, but is most commonly found in the pelvis, thigh bone (femur) and shin bone (tibia).
The exact cause of this type of bone cancer is not known. However, changes in the cell’s chromosomes are thought to be the reason for Ewing sarcoma formation. These changes occur after birth and are not inherited.
Symptoms of bone cancer include persistent pain, swelling, or tenderness in the affected area. Bone pain may become worse during exercise or at night. Bones may become weak and can fracture. Tumors affecting the legs may cause a limp.
Your doctor will ask about your family and medical history, perform a physical examination and may order certain procedures and tests to diagnose sarcoma. These may include:
X-rays: X-rays of the affected area may be taken to find out the location, size and shape of the tumor.
Computerized tomography (CT) scans: This is a procedure that uses cross-sectional images to evaluate whether the tumor has spread.
Magnetic resonance imaging (MRI): This test uses a magnetic field to create detailed images of the body.
Positron emission tomography (PET): A PET scan can show whether cancer has spread to other parts of the body.
Biopsy: A sample of tissue is removed from the tumor for laboratory testing to examine whether the tissue is cancerous.
Treatment of bone cancer is decided based upon the type, nature, extent of its spread, and the patient’s general health. Treatment options include surgery, radiation therapy, and chemotherapy.
Chemotherapy uses drugs or medications to destroy the cancer cells. It is administered prior to surgery to kill the tumor cells and shrink the tumor. It is also prescribed after surgery to kill any remaining cancer cells and prevent recurrence.
Surgical treatment involves removing the cancerous tissue and consists of techniques such as limb-salvage, or limb sparing surgery or in rare cases, amputation.
Limb-salvage surgery removes the cancerous bone and the affected muscle. The gap is filled with a bone graft or a specially designed metal prosthesis.
Amputation, the removal of a part or whole of the affected limb, may also be required if the cancer has spread from the bone to the surrounding blood vessels.
In certain cases radiation therapy that harnesses high energy radiation is indicated as treatment to destroy the cancer cells.
Adverse effects of cancer therapy
Some side effects of chemotherapy include fever, nausea, hair loss, and mouth sores. Medications can be given to help control these side effects.
Common side effects of radiotherapy include skin problems, decreased joint flexion, injury to surrounding organs, delayed wound healing, reduced bone growth in youth, risk of new cancer incidence and chronic swelling.
The risks and complications associated with surgery include anesthesia complications, infection, bleeding, and reoccurrence of the cancer.
After surgery, the patient is instructed on how much weight their reconstructed limb can safely handle. Rehabilitation including physical therapy can help resumption of day-to-day activities. Your physical therapist recommends specific exercises to help restore strength and improve range of motion. Strenuous or athletic activities that place too much stress on the reconstructed limb should be avoided.
Bone sarcomas can affect both children and adults. If anyone in the family, irrespective of the age, experiences bone pain or suspects bone cancer, an appointment should be made with your doctor for further evaluation.