cancer by site and during special times

Bone Cancer
RAFAEL S. CLAUDIO, MD, FPSO, AGNES E. GOROSPE, MD, FPSO AND RICHARD S. ROTOR, MD, FPSO

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ones are vital parts of the human body. They support and protect internal organs. They serve as attachments for muscles, tendons, and ligaments to produce motion. Their marrow, too, are vital as it produces and stores blood cells. Primary bone cancer refers to cancers which start in the bone. Most primary bone cancers are called sarcomas. Sarcomas may also be found in the muscles, blood vessels, nerves and fatty tissues. Secondary bone cancer (metastatic bone disease) occurs when cancer cells have spread to the bones from other parts of the body. Almost all types of cancer can spread to the bone. Bone metastases are particularly common in people with breast, lung, thyroid, kidney or prostate cancer. Bone metastases can be at multiple sites and cause severe pain and disability, especially when they a ect the hips, thighs and legs. Secondary bone cancer is the most common type of bone cancer. In contrast, primary bone cancer is a rare type of cancer. It accounts for only about 0.2% of all cancers. In the Philippines, no detailed national statistics are available, but in one institution and for osteosarcoma, a form of childhood bone cancer, it was reported in 2003 to be about 52 cases per year, or about 1 per week. In the UK, there are only about 550 cases of bone cancer reported annually. In the USA in 2002, there were 2400 cases of primary bone cancer incidence reported. HOW DO WE KNOW ONE HAS BONE CANCER? Symptoms of bone cancer vary from person to person, on the location, type and size of the cancer. The most common symptoms are pain and swelling or a mass/tenderness in the a ected area. In a study based on a Philippine government hospital, majority (70%) of patients initially seek consult due to a mass. The pain experienced is usually due to compression of a nerve or muscle by the mass. Sometimes, bone cancer can interfere with movement and weaken the bones, occasionally leading to a fracture. Other symptoms of cancer may include fatigue, fever, weight loss and anemia. None of these symptoms, however, is a denite sign of cancer. WHAT ARE THE CAUSES AND ITS RISK FACTORS? Scientists have not yet pointed to a single cause for bone cancer. There are, however, a number of factors which may put a person at increased risk. 1. Primary bone cancer is more common in children and young adults. Secondary bone cancer is more frequent among the over-40 years. 2. Patients who have undergone radiotherapy or chemotherapy for other conditions are also at risk.

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3. Other risk factors include trauma, Paget's disease wherein the bones enlarge and weaken and multiple overgrowth of bone tissue (multiple exostoses). 4. A small number of bone cancers are hereditary. People who have Li-Fraumeni syndrome, a condition caused by a rare genetic defect, have an increased risk of developing bone cancer. Retinoblastoma, a rare inherited cancer of the eye in children, is also associated with and increase the risk of developing bone cancer. HOW IS IT DIAGNOSED? Once your doctor suspects the presence of a bone tumor, he will inquire about your personal medical and family history. A complete physical and medical examination will also be performed. This may include a blood test as bone tumors can be associated with increased levels of certain products in the blood. One blood examination that may be requested determines the level of an enzyme called alkaline phosphatase. Elevated levels may be seen in patients with osteosarcoma, bone cancer that a ects the femura bone of the legand is most common in kids. However, elevated levels may also be seen in actively growing children and during fracture healing. The doctor may also recommend x-rays of the a ected bone. These will show the location, size and shape of the tumor. If x-rays hint towards a tumor, your doctor may recommend special imaging tests like a computerized axial tomography (CAT or CT) scan, magnetic resonance imaging (MRI), bone scan or angiogram (blood-vessel x-ray). These will help your doctor further understand the extent and activity of the tumor. A biopsy or removal of sample tissue for examination may then be recommended by your doctor. A sample will be obtained either through a hollow needle-like instrument or through incision to cut into the tumor. Biopsies are best done by orthopedic oncologists who are trained and experienced in the diagnosis and treatment of bone cancer. This will be then be studied by a pathologist, the medical diagnosis specialist, to determine whether cancer is present. HOW DO WE KNOW YOU ARE IN THE EARLY OR LATE STAGE OF BONE CANCER? Your doctor makes use of a staging system to help him in the treatment process as well as in proper documentation. This staging is based on whether or not the tumor has gone beyond the bone, whether the tumor
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cells are aggressive or not and whether metastases exist. Bone Cancer can be staged into the following: Stage 1 - Low grade tumor without metastasis Stage 2 - High grade tumor without metastasis Stage 3 - Any grade of tumor with metastasis Low grade tumors spread outward from the original tumor but, generally do not metastasize. High grade tumors grow rapidly to surrounding tissues or may metastasize to other parts of the body. HOW IS IT TREATED? For patients with benign or harmless bone tumors, treatment may consist of simple observation, casting or even surgery if there is a break in the bone. For patients diagnosed with bone cancer or sarcomas, however, treatment depends on the type of cancer, size, location, stage of the cancer and patients age and general medical condition. Surgery aims to remove the cancer while preserving much healthy bone as possible. This is called limb salvage surgery. An orthopedic surgeon/oncologist will remove the a ected muscle and bone and reconstruct your limb with the use of metal implants, bone from your own body (autograft) or frozen bone from a bone bank (allograft). Amputation, the removal of the whole limb, is still done locally for patients with bone cancer whose tumors are very large or have invaded on a major blood vessel. Many articial limbs for both the upper and lower extremity are available and manufactured locally. In some cases, chemotherapy or radiation therapy may be given before surgery to shrink the cancer, making it easier to remove. Patients may also receive chemotherapy after surgery to destroy any remaining cancer cells. Treatment for bone metastases varies. It will depend on the type of cancer, extent of the spread of metastases, prior treatment and other factors. In general terms, treatment of bone metastases is palliative or supportive (relief of symptoms). This includes treating the patient when he has a fracture due to the metastasis. For those people with advanced stages of cancer, since there may be no known e ective cure, treatment is directed at prolonging life and relieving the symptoms of bone metastases (eg., pain relief), thus improving the quality of life. In certain cases where there is a chance of cure, the treatment may include chemotherapy, radiotherapy and possibly bone marrow/stem cell transplantation.

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WHAT ARE THE CHANCES OF SURVIVAL? Overall, the chance of recovery (prognosis) for bone cancers has improved signicantly since the development of modern chemotherapy. Before the advent of chemotherapy, survival for patients with osteosarcoma was less than 20 percent. Nowadays, survival rates both internationally and locally range from 60-70 percent. Prognosis depends on whether the cancer has spread, the type of bone cancer, the size of the tumor, location, the persons general health and other factors. Of equal importance is how much of the main tumor can be removed/destroyed by surgery and/or radiotherapy and how the tumor responds to chemotherapy. FREQUENTLY ASKED QUESTIONS 1. Are all tumors of bone cancerous? There are two general types of bone tumors. Benign (non-cancerous) bone tumors do not spread to other parts of the body and are rarely life threatening. Malignant (cancerous) bone tumors, on the other hand, are capable of spreading particularly to the lung. Benign tumors are more common than the malignant type. 2.What are the types of bone cancer? The most common type of primary bone cancer is osteosarcoma, which develops in growing bones in children between the ages of 10-20. Another type, chondrosarcoma, arises from cartilage, and occurs more in adults. Ewings sarcoma, another form of bone cancer in children, arises from immature nerve tissue in bone marrow. Secondary bone cancer or bone metastasis, meanwhile, is the most common form of bone cancer 3. Will I still be able to walk after surgery for a tumor in my knee? Depending on the type of surgery, you will still be able to walk after a tumor around the knee joint is removed. You may not be allowed to bear weight on your operated limb immediately after the surgery, but may be allowed to walk with crutches. However, if your knee has been xed in a straight position, bending it will not be possible. You will still be able to walk but with a slight limp, though.

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4. Will I be able to ride public transportation like tricycles, jeeps and buses? If after surgery your knee is xed in a straight position, you will have some di culty getting on jeeps and buses. In addition, you will need to sit near the aisle in buses or even airplanes since you will need extra space for your straight limb. 5. After an amputation, will I still be able to walk or use my arm? Amputation does not mean you will not be able to walk or use your arm. Articial limbs are readily available locally. Depending on the area of amputation, various articial limbs (prosthesis) are manufactured locally. There are patients who can run and jump with the use of their prosthesis. However, for the arms, the prosthesis will most often be more for appearance than for function. Patients are taught how to use the other arm to compensate for the loss. If you are right handed and your right arm is amputated, you will be taught and trained to use your left arm and hand. 6. If my tumor is in the muscle, fat or nerve, what can I expect after surgery? After removal of the tumor, some weakness of the limb may be expected. Nevertheless, with physical therapy, some strength may be regained and the limb will be functional again. If a nerve has been removed, aside from weakness, some numbness in the area will be experienced. The weakness, or at times, paralysis of some muscles may be compensated with the use of a brace (orthosis) to help you move the limb.

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