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My Child Has:
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Adamantinoma
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An adamantinoma is a slow growing cancerous bone tumor that is most often found in the shin bone (tibia) or the jaw bone (mandible). Occasionally, these tumors are found in the forearm, hands or feet. It is a rare tumor, occurring primarily in males between the ages of 10-30, usually after skeletal maturity is reached.
In about 20 percent of cases, adamantinoma cancer cells spread (metastasize) to other parts of the body, usually to the lungs or nearby lymph nodes.
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The cause of adamantinoma is unknown. Patients with adamantinoma usually have a history of trauma to the affected bone, but it is unclear whether trauma is involved with formation of the tumor or the formation of the tumor leaves the bone weak and susceptible to injury.
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The following are the most common symptoms of an adamantinoma. 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
- increased pain with activity or lifting
- limping
- decreased movement of the affected limb
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The symptoms may have been present over a short period of time or may have been occurring for six months or more. Often, an injury brings a child into a medical facility, where an x-ray may indicate suspicious bone lesions.
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In addition to a complete medical history and physical examination, diagnostic procedures for adamantinoma include (not necessarily in this order):
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- biopsy of the tumor - a small sample of the tumor is surgically removed and analyzed under a microscope. The cellular appearance of the tumor under a microscope provides the information doctors need to definitively distinguish adamantinoma from other bone tumors. Sometimes multiple biopsies are needed to diagnose this condition.
- 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)
- Multiple imagine studies of the tumor and sites of possible metastasis, such as:
- x-rays - a diagnostic test which uses invisible electromagnetic energy beams to produce images of internal tissues, bones and organs onto films. X-rays are very useful in the diagnosis of bone tumors and frequently allow the physician to distinguish benign from malignant bone tumors. An x-ray is typically the first diagnostic study and it often gives the doctor information regarding the need for further testing.
- 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 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. They are used primarily to assess the chest and lung for metastatic tumors.
- Bone scans - a nuclear imaging method to evaluate any degenerative and/or arthritic changes in the joints; to detect bone diseases and tumors; to determine the cause of bone pain or inflammation.
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Once adamantinoma is diagnosed, doctors will want to know how far the tumor has progressed. Staging the tumor indicates how far the tumor has spread from its original location. The stage of a tumor suggests which form of treatment is most appropriate, and gives some indication of your child's prognosis.
More often, adamantinoma does not spread beyond the bone where it arose or beyond nearby tissues. Although, in 20 percent of cases, the tumor becomes metastatic, meaning it spreads to lungs, lymph nodes, bones other than the bone that the tumor originated in, or to other organs or structures of the body.
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Specific treatment for adamantinoma will be determined by your child's physician based on:
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- Your child's age
- Extent of the disease
- Your child's tolerance for specific medications, procedures, or therapies
- How your child's doctor expects the disease may progress
- Your opinion or preference
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Treatment for most bone tumors can include a combination of surgery, chemotherapy and/or radiation. However, adamantinomas have proven to be highly resistant to both chemotherapy and radiation, making surgery alone the option of choice.
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Surgery for adamantinoma involves the biopsy, surgical removal of the tumor, bone/skin grafts, limb salvage procedures, amputation, and/or reconstruction, all performed by an orthopaedic surgeon. The type of surgery will depend on the size and location of the tumor, and whether the cancer has spread.
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- Limb-salvage surgery: Usually adamantinoma can be treated by removing the involved segment of bone and replacing it with a bone graft either from another part of the body or from a bone bank (allograft). Some centers have use bone transport (Ilizarov technique) to reconstruct the bony defect after resection. With both techniques, it is usually possible to achieve excellent functional results and good tumor control. Rarely, amputation will be necessary.
- Amputation: Rarely, your child's orthopaedic surgeon may determine that the tumor cannot be removed because, for example, it involves the nerves and blood vessels. In this case, amputation may be the only option. During the operation, doctors ensure that muscles and skin form a cuff around the amputated bone. A cast is applied in the operating room which permits a temporary artificial leg (prosthesis) to be applied during the first few post-operative days for walking. Crutches are used for several weeks. As the swelling decreases (10 to 14 days) the patient is fitted for a plastic, temporary socket and prosthesis, which is used for 3 to 4 months until the stump is healed sufficiently to accept a permanent artificial leg. The advantages of an amputation are that it is a simple operation with minimal chances of surgical complication and it definitively removes the local tumor. The functional outcome is good with the modern prostheses available today and with "immediate-fit" prostheses applied in the operating room. Although the patient will probably have a limp with above-the-knee amputations, the procedure is functional and stable. He/she will be able to walk, climb stairs, swim (with the prosthesis on or off) and participate in many sports such as skiing, basketball, baseball, and tennis although running will be limited. The functional limitations are left to the imagination and determination of the patient.
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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 determined by your child's physician and other members of you care team to monitor ongoing response to treatment and possible late effects of treatment. Treatment options will vary greatly, depending on your child's individual situation. Your child's physician and other members of your care team will discuss these with you in-depth.
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Prognosis for adamantinoma 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 structures 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 adamantinoma. Second malignancies can occur in some survivors of this disease. New methods are continually being discovered to improve treatment.
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