 Chondrosarcoma- Head and Neck
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Chondrosarcoma is a type of cancer that resembles cartilage, the smooth, flexible, connective tissue that coats the ends of bones and forms joints. Chondrosarcomas occur primarily in adults, is rarely encountered during the adolescent years and almost never affects young children. It can be a primary tumor, meaning that it is the original tumor and is not the result from a tumor that spread or it may develop secondary in a pre-existing benign cartilage tumor).
A secondary chondrosarcoma can result from a malignant change in benign tumors such as an osteochondroma (a tumor made of bone and cartilage that occurs near the end of a long bone) or enchondroma (a benign cartilage tumor that is usually found inside the long bones).
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Chondrosarcoma occurs when a single abnormal cartilage cell divides out of control. Scientists are not certain what causes this to occur, although several factors may put a person at risk. For instance, this cancer can occur in patients with hereditary abnormalities such as Ollier's disease, Maffuci's syndrome or exostoses (bone growths), although this is rarely the case in children.
There is some relationship between chondrosarcoma and repeated trauma to the affected area, but it is unclear whether injury puts a person at risk for this type of cancer, or the cancer itself makes the area weaker, thus making injury more likely.
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The following are the most common symptoms of chondrosarcoma. However, each child may experience symptoms differently. Symptoms may include, but are not limited to the following:
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- Pain (sharp or dull) at the site of the tumor
- Swelling and/or redness at the site of the tumor
- Enlargement or growing of a pre-existing exostosis (osteochondroma)
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The symptoms of chondrosarcoma may resemble other conditions or medical problems. Always consult your child's physician for a diagnosis.
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In addition to a complete medical history and physical examination of your child, diagnostic procedures for chondrosarcoma may include:
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- Multiple imaging studies of the tumor and sites of possible metastasis, such as
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- x-ray - a diagnostic test that uses invisible electromagnetic energy beams to produce images of internal tissues, bones, and organs onto film. X-rays are very useful in the diagnosis of bone tumors and frequently allow physicians to distinguish benign from malignant bone tumors. It is the first diagnostic study, and often gives the doctor information regarding the need for further testing
- bone scans - a nuclear imaging method used to detect bone and metastatic tumors. Bone scans can determine if there are abnormalities in other bones. This test does not distinguish between tumor, infection or fractures.
- magnetic resonance imaging (MRI) - a diagnostic procedure that uses a combination of large magnets, radiofrequencies, and a computer to produce detailed images of organs and structures within the body. This test outlines the extent of the tumor within the bone and joint and the relationship of the tumor to the muscles, nerves and blood vessels.
- computerized tomography scan (also called a CT or CAT scan) - a diagnostic imaging procedure that uses a combination of x-rays and computer technology to produce cross-sectional images (often called slices), both horizontally and vertically, of the body. A CT scan shows detailed images of any part of the body, including the bones, muscles, fat, and organs. CT scans are more detailed than general x-rays. It is used primarily to assess the chest and lung for metastatic tumors.
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- complete blood count (CBC) - a measurement of size, number, and maturity of different blood cells in a specific volume of blood
- blood tests (including blood chemistries)
- biopsy of the tumor - a small tissue sample of the tumor is surgically removed and examined under a microscope. This procedure enables physicians to establish the diagnosis and distinguishes chondrosarcoma from other sarcomas. Biopsy also helps doctors to determine how aggressive the tumor is.
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Once chondrosarcoma has been diagnosed, the tumor is staged. This process indicates how far the tumor has spread from its original location. The stage of the tumor and grade of the tumor (how aggressive the tumor is) suggests which form of treatment is most appropriate, and gives some indication of the prognosis.
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Specific treatment for chondrosarcoma will be determined by your child's physician based on:
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- your child's age, overall health, and medical history
- extent and location of the disease
- your child's tolerance for specific medications, procedures, or therapies
- how your child's physician expects the disease may progress
- your opinion or preference
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Treatment for a low grade chondrosarcoma involves an operation to remove the tumor. (See "Surgery" below) This kind of surgery alone is usually sufficient to cure these less-invasive tumors.
A rapidly growing, high grade chondrosarcoma is treated in similar fashion to a high-grade osteosarcoma, and may include, surgery combined with other forms of therapy, which are discussed below.
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Surgery for chondrosarcoma involves the biopsy, surgical removal of the tumor,and/or bone/skin grafts.The type of surgery will depend on the size and location of the tumor, and whether the cancer has spread.
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This therapy is rarely used in chondrosarcoma except for extremely aggressive forms of the disease. Chemotherapy is a drug treatment that works by interfering with the cancer cell's ability to grow or reproduce. Different groups of drugs work in different ways to fight cancer cells and shrink tumors. Chemotherapy may be used alone for some types of cancer or in conjunction with other therapy such as radiation or surgery. Often, a combination of chemotherapy drugs is used to fight a specific cancer. Certain chemotherapy drugs may be given in a specific order depending on the type of cancer it is being used to treat. While chemotherapy can be quite effective in treating certain cancers, the agents do not differentiate normal healthy cells from cancer cells. Because of this, there can be many adverse side effects during treatment. Being able to anticipate these side effects can help the care team, parents, and child prepare, and, in some cases, prevent these symptoms from occurring, if possible.
Chemotherapy is systemic treatment, meaning it is introduced to the bloodstream and travels throughout the body to kill cancer cells. Chemotherapy can be given:
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- as a pill to swallow
- as an injection into the muscle or fat tissue
- intravenously (directly to the bloodstream; also called IV)
- intrathecally - chemotherapy given directly into the spinal column with a needle
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Rehabiliation includes physical and occupational therapy along with psychosocial adapting
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Supportive care refers to any type of treatment to prevent and treat infections, side effects of treatments, and complications, and to keep your child comfortable during treatment
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A schedule of follow-up care will be determined by your child's physician and other members of your care team to monitor ongoing response to treatment and possible late effects of treatment
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Prognosis for chondrosarcoma greatly depends on:
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- the extent of the disease
- the size and location of the tumor
- Presence or absence of metastasis.
- the tumor's response to therapy.
- the age and overall health of the child
- your child's tolerance of specific medications, procedures, or therapies.
- new developments in treatment
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As with any cancer, prognosis and long-term survival can vary greatly from child to child. Every child is unique and treatment and prognosis is structured around the child's needs. Prompt medical attention and aggressive therapy are important for the best prognosis. Continuous follow-up care is essential for a child diagnosed with chondrosarcoma. Side effects of and chemotherapy, as well as second malignancies, can occur in survivors of more aggressive forms of chondrosarcoma. New methods are continually being discovered to improve treatment and to decrease side effects.
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