Radiotherapy treatment of musculoskeletal tumor
Skeletal tumors - malignant and benign
Sceletal tumors, if they are formated in the bone they can be malignant and benign and those are called PRIMARY TUMORS, while the SECONDARY tumors are those who arise from metastasis of other diseased tissue. Malignant tumors are progressive and they frequently relapse ( they will apeear again in the same spot from which the tumor has already been removed ), and they often metastasize to other tissue, and they are transmitted through the blood and lymph.
Benign tumors don't have any of the characteristics of malignant tumors, no recurrences or metastases and are built of tissue that is similar to normal tissue. It is very important to detect tumor at an early stage of progress, because that is the time when the possibility to cure is a lot higher than when the tumor is at an advanced stage.
The most common treatment is surgical therapy, or surgical removal of the bone that is affected by the tumor, and in severe cases (when the tumor is malignant and indicates invasion to the surrounding tissue), the removal of the entire limb. The treatments which are also used are chemotherapy and radiation therapy, although the tumors of bone are often insensitive to these therapies.
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PRIMARY BENIGN TUMOR - PRIMARY MALIGNANT TUMOR - METASTATIC TUMORS
PRIMARY BENIGN TUMORS - these tumors are easily removed from the bone and they may be replaced by the patient's bone tissue or preserved bone tissue. Benign tumors are chondroma or enchondroma. Chondroma usually occur in the bones of the arms and legs, and it is often revealed by fractures of these bones caused by the thinness of the bone walls. Chondroma are the most similiar to the cysts, but they differ from cysts in content. The most common benign bone tumors are exostoses (growths wrapped cartilage). In any case, benign tumors are curable, the prognosis for most patients is very good.
PRIMARY MALIGNANT TUMOR - Some of the primary malignant tumors are: lymphogranuloma malignant, malignant neurinoma, lymphosarcoma, plasmacytoma, and osteosarcoma. There are quite a few types of primary malignant tumors. Course and diagnosis depend on the tissue in which the tumor is. The disease is diagnosed based on examination of the patient, clinical radiographic images, microscopic histological analyses of the tumor and macroscopic appearance. Based on the diagnosis, a decision is made whether it is necessary to remove only part of the bone that is affected or the whole limb must be removed. If the disease is diagnosed at an early stage, then there are greater chances for success of therapy.
METASTATIC TUMOR - malignant tumors that develop in other tissues and organs can metastasize in bones. Metastatic bone tumors are the most common cancers (tumors composed of epithelial cells). Metastasis to the bone extends through the blood, and causes the creation of secondary tumors. Bone structure gets weaker, often leading to pathological fractures, and thus reveal the presence of tumors.
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Osteosarcoma is the most common type of bone tumor, representing approximately 35% of primary malignant bone tumors. Mostly affects young people between 10 and 25 years, when bones are growing extensively, and it affects mostly men. It most often occurs in the area around the knee and at the end of long bones of the arms and legs.
Osteosarcoma is characterized by the fact that tumor cells directly produce osteoid (a mixture of proteins which are secreted by osteoblasts and that becomes a bone when the minerals are disposed in it). Sometimes osteosarcoma can occur in older people, and then it is most associated with Paget's disease and radiation. At the time of diagnosis 15-20 percent of patients had metastases to the lungs or other bones.
People who get a later diagnosis of osteosarcoma have following symptoms : pain, sensitivity to touch, swollen bones, limited movement, sudden fracture in otherwise healthy people.
Not to waste precious time on the wrong diagnosis and treatments, it is necessary to x-ray the area affected by osteosarcoma. Radiographic confirmation of the existence of the tumor is followed by computerized tomography (CT), magnetic resonance imaging scan (MRI) or positron emission tomography (PET). Also, the specialist may perform a biopsy to determine cell type. Location, size and extent of tumor affects the selection and the outcome of treatment.
The exact cause of these tumors is not fully known, but a very large number of factors are such as exposure to radiation, intense bone growth, as well as genetic predisposition (occurs more frequently in certain conditions and syndromes such as Li-Fraumeni syndrome, Rotmund-Thompson syndrome ).
HOW TO SET A DIAGNOSIS OF OSTEOSARCOMA?
Medical history with a clinical picture can help in making the diagnosis, but is not sufficient for their setup and definitely some additional tests have to be taken. In laboratory analyses are often found elevated alkaline phosphatase and lactate dehydrogenase (in patients with elevated alkaline phosphatase levels have the greater the likelihood of finding lung metastases).
It is always necessary to make a complete biochemical, electrolyte analysis, to estimate the parameters of liver and kidney function. Diagnosis is based on the X-ray image of bones from multiple projections, scanners (computerized tomography) and nuclear magnetic resonance. Often is used bone scintigraphy. The definitive diagnosis is made on the basis of histopathological analysis of biopsy samples obtained after the operation.
For the treatment of osteosarcoma are responsible expert clinicians, orthopedic specialists and oncologists. Chemotherapy can lower the tumor and treat possible metastasis, such as the metastasis on lungs. After that, the surgery of the limb or affected area may be followed, in order to prevent amputation. Reconstructive surgery may also be an option, and may include a series of operations to reimplant tendon or to implant prosthesis. In some cases, chemotherapy after surgery is not possible. In others, there are types of operations that are possible, but they can weaken the body. In such situations radiation is considered, especially if the biopsy showed that it is a specific type of cell. It must be taken care of possible complications during treatment, as well as side effects such as wound infection, pain and nausea.
Prognostic factors depend on the localization of the primary tumor. Tumors that have a poor outcome are tumors of collar bone, scapula, clavicle, ribs, vertebrae and pelvis, proximal parts of the extremities. In the treatment is used SURGICAL THERAPY, RADIATION THERAPY AND IMMUNOTHERAPY. After a bone biopsy current treatment programs recommend preoperative chemotherapy that reduces the tumor. After that comes surgery and chemotherapy again. It is recommended a combination of high doses of calcium folinate with Metotrexat, adriblastin, high doses cisplatinuma and ifosfamide or high dose methotrexate in combination adriblastina, cyclophosphamide, bleomycin and actinomycin.
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Osteosarcoma - Learn more about this disease
Liposarcoma is a malignant tumor of adipose tissue. So, it's a tumor that may give distant metastases. It is the most common form of soft tissue sarcoma that occurs in adults. Fortunately, lipomas rarely malignantly alter in liposarcoma. In the literature it is mentioned that the percentage is about 0.1%. These are slow-growing changes that may exist for a long time without any symptoms. The most common location of metastasis of these tumors are the lungs and liver. There are five categories of liposarcoma : well differentiated, pleomorphic , myxoid, dedifferentiated and round cell. The anatomical distribution of these tumors is related to the differentiation of their cells and well differentiated tumors are found mostly in the deep tissues of the extremities and retroperiotoneum, while other kinds are commonly found in the extremities. The exact cause is not expressed in detail, and some authors suggest a certain degree of genetic predisposition with the effects of trauma.
How are liposarcoma manifested :
-These tumors are rarely localized in the neck and the head, and more often found on the extremities and in the retroperitoneal space
-commonly found on the thighs and groin
- usually develop from deep tissue, and slightly less often from subcutaneous tissue
-most patients have no symptoms until the tumor ‘s size doesn’t cause disturbances (pain, vulnerability and functional disability)
-these tumors show slow growth and often metastasize to the liver and lungs
- common nonspecific symptoms are such as fatigue, loss of appetite, vomiting, weight loss
-it is possible occurrence of increased sensitivity, pain, swelling and varicose veins in the extremities
- If they are large and interfere with their compression to the functions of internal organs, that may be associated with thrombosis, ileus, volvulus, intussusception, constipation
-they can grow a lot until they reach the time of diagnosis.
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HOW TO SET A DIAGNOSIS OF LIPOSARCOMA?
Anamnesis with clinical and objective examination is enough to doubt the existence of a tumor, but additional tests must be taken. Abdominal ultrasound and soft tissue ultrasound is sufficient to determine the size, shape and location of tumor, and is used as a supplement to scan (computed tomography) or MRI.
An X-ray of lungs is used to evaluate the possible presence of pulmonary metastases. PET (positron emission tomography) with radioactively labeled fluoro glucose has its place in the diagnosis. A definite diagnosis can only be based on histopathological analysis of samples taken by biopsy or during surgical treatment.
Treatment involves radical surgical removal of changes (if possible) with Radiation Therapy. Unfortunately, only undifferentiated tumors show mild chemosensitivity, which means that for other forms of tumors chemotherapy use has no excuse. Prognosis depends on the type of liposarcoma. Well-differentiated and myxoid type have a good prognosis, they rarely metastasize, whereas undifferentiated and round cell liposarcoma relapse and quickly give metastases.
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Chondrosarcomas tumors consist of malignant chondrocytes. Many Chondrosarcomas are slow-growing tumors or with low-grade malignancy, which is often treated with surgical procedures. However, some of them have a high degree of malignancy (fast-growing) and are more likely to spread (metastasize). Biopsy is necessary to diagnose tumors. Chondrosarcomas must be completely surgically removed because they are not sensitive to chemotherapy and radiation. Amputation of an arm or leg is rarely needed. More than 75% of people who have chondrosarcoma survive.
Fibrosarcoma is a rare bone tumor in children. Usually affects the soft tissues of the lower leg. It grows slowly and relapses late. On the radiograph is osteolytic area of blurred boundaries, and clinical - a tough infiltrate surrounding the affected bone. One third of patients occurs with pathological fracture.
Chordoma is a very rare tumor, with an average survival of 6 years after diagnosis. More common in men, after thirty years of age. Predominantly affects the upper and lower ends of the spine.
Ewing's sarcoma is the most aggressive tumor of bone. More common in boys in the age group between 4 and 15 years, and is extremely rare after 30 years. It typically occurs in the middle of the long bones of the arms and legs. On x-ray images you can see symmetric thickening of spindly bones. Bone is in places mottled with osteolytic areas, and in some places there is thickening of bone. The picture is very unclear, whole bone acts as it is often described as "chewed by moths." Three-year survival rate is about 65%. The prognosis is significantly worse in patients with metastases to the lungs or other parts of the body.
Malignant lymphoma of bone (reticulo cellular sarcoma) occurs in adults in their 40s and their 50-year life. It can occur in any bone. The tumor usually causes pain and swelling, and damaged bone is prone to fracture. Treatment usually requires a combination of chemotherapy and radiation, which appears to be as effective as surgical removal of the tumor. Amputation is rarely necessary.
Multiple myeloma (also known as a plasmacytoma or Kahler's disease) is a malignant change in plasma cells in which plasma cells are uncontrolled produced in the bone marrow. Pathologically altered plasma cells produce antibodies or only parts of an antibody - called paraprotein. These antibodies are only one kind (eg IgA),and they are produced by the so-called clone of plasma cells, and are also called monoclonal protein. Paraprotein production prevents other normal antibodies, that is why the patient becomes susceptible to infections. It is slowed the growth of healthy blood-forming cells in the bone marrow. The lack of healthy red and white blood cells produces fatigue, weakness, headache, and increased propensity to infection. Reproduction of myeloma cells in the bone marrow, their interaction with the so-called stroma (stroma, the supporting tissue of an organ or tumor) and activation of killer cells leads to enhanced activity of osteoclasts. Osteoblasts are cells that reduce bone mass and cause bone defects, which can lead to fractures and pain. While biodegrading of the bone tissue calcium is released. Increase in the amount of calcium in the blood leads to various complications, such as kidney damage, fatigue, confusion, heart rhythm disturbances, nausea and vomiting. There are known various forms of multiple myeloma. They vary in structure produced antibodies.
For a positive diagnosis must be satisfied at least two of the following criteria:
- proportion of plasma cells in the bone marrow sample is greater than 10%
- Damage of bone is specific for myeloma (which looks like punched holes in the skeletal system), or a decrease in bone density (osteoporosis) and fractures. However, none of these bone damages isn’t specific just for myeloma
- It is possible to detect more than 3 grams of paraprotein in 100 ml of blood and / or paraprotein is found in urine and / or a decrease in bone thickness
In addition to blood tests and urine tests, x-ray, nuclear magnetic resonance or computed tomography are important tests for detecting osteolysis (points at which bone mass is reduced). Puncture or bone marrow sample for analysis is the basis for setting diagnosis of multiple myeloma.
The main goal of any therapy is to significantly suppress the disease. In multiple myeloma permanent cure is possible only in those patients in whom the disease affected only a limited area (extramedullary plasmacytoma and solitary plasmacytoma) and in younger patients who are treated with allogeneic bone marrow transplant (from a donor). In addition to this, multiple myeloma is incurable with current methods of therapy. Therefore, the goal of therapy is prolongation of life and raising its quality.
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TREATMENT OPTIONS FOR MULTIPLE MYELOMA
- Chemotherapy - chemotherapeutic agents are substances which inhibit the growth of tumor cells. Opposite to other malignant tumors that require immediate initiation of treatment, chemotherapy for multiple myeloma is not necessarily justified by the diagnosis. However chemotherapy should be initiated:
- If the symptoms are such as reduced physical fitness, fatigue, weight loss and pain in the bones,
- If there is evidence of decline in renal function,
- At elevated calcium levels in the blood or
- If there are signs of anemia.
- RADIATION THERAPY – The main goal in radiation is to prevent duplication of malignant cells and to prevent further growth of the tumor. Unlike many tumors, irradiated healthy cells have the ability to repair damage caused by radiation, with the result that the radiation has a much greater effect on the tumor than on healthy organs in the surrounding area. In multiple myeloma, radiation therapy is performed primarily to reduce the pain in the bones. In addition, radiation therapy can prevent fractures in statically loaded parts of the skeleton. Also applying radiation can stabilize existing fractures. It is important to know that no radioactive substance doesn’t penetrate into the body during radiation therapy.
- Treatment with interferon - A new therapeutic approach in the treatment of myeloma is the use of thalidomide. Scientists have discovered that thalidomide can inhibit the formation of new blood vessels. Thalidomide is often used in the treatment of newly diagnosed patients, patients with relapsed disease and the disease which has been shown to be resistant to standard treatment. For patients who have successfully completed another initial therapy is used as maintenance therapy.
- BORTEZOMIB TREATMENT - The development and collapse of signaling proteins are equally important for the survival of tumor cells, as well as the reproduction of cells, adhesion of myeloma cells (where they "stick" to the bone marrow, they can’t come off and resume normal maturation, and thus displace normal bone marrow cells) and the formation of new blood vessels. Creating of new cells is called angionesis, which requires oxygen, nutrients and minerals. New drugs such as Bortezomib prevent this process in myeloma cells so that they can’t grow - anti-angionesis.
SYMPTOMS OF BONE TUMOR
Symptoms of bone tumors in children are different, depending on the size and location of the tumor. The most common symptom is pain, which is initially present at night or in certain activities. In most patients, the pain becomes stronger with time. Depending on the rate of tumor growth, the pain can last for weeks, months or years before seeking medical help. Sometimes you can see the swelling. If parts of bone are destroyed by a tumor, fracture can occur spontaneously or after minimal trauma (called a pathological fracture). Common symptoms of the disease, as fever, fatigue, malaise, or continuous and unexplained weight loss are more common in patients with metastatic disease.
DIAGNOSIS OF BONE TUMORS
The doctor will first take a detailed medical history. Interpretation of symptoms may help suspecting tumor of bone. Furthermore, it is important a complete physical examination, including muscle strength and reflexes. Routine blood tests must be done, which in patients with bone tumors aren't specific.
X-RAY - Classis footage of two courses covering the whole affected bone. It is simple, cheap, available in smaller institutions with the minimum of radiation and doesn't require general anesthesia for a child. Radiographic changes help to determine the type of tumor and whether the tumor is benign or malignant. Benign tumors are significantly more likely to have a proper straight line, while malignant tumors have irregular line. Benign tumors usually grow slowly and the bone has enough time to limit tumor with normal bone. On the other side, malignant tumor is characterized by rapid growth, which doesn't give time to normal bone to limit tumor. Symmetry is crucial in assessing the findings. When the x-ray shows something suspicious, a doctor can compare the image with the other side. Besides, it is pointed out that many of malignant bone tumors have characteristic radiographic findings. Patients who have malignant bone tumor it is mandatory to have the image of lungs for detecting any metastases.
Computed tomography (CT) is an advanced search for three-dimensional visualization of bone, which better identifies tumor and provides additional information about its size and location. The main disadvantages are higher doses of radiation and price. Furthermore, in young children CT scan must be performed under general anesthesia. These disadvantages, however, are negligible compared to the importance of early diagnosis of malignant tumors of children.
Magnetic resonance imaging (MRI) is another advanced radiologic examinations, which provides more detailed information than computed tomography of the soft tissues surrounding the bone, including muscles, tendons, ligaments, nerves and blood vessels. In addition, better reflects the eventual penetration of the tumor through the compact part of the bone and surrounding soft tissues. MRI has no ionizing radiation. The main disadvantage is the high cost and limited availability.Ultrasound can analyze only soft tissue.
Scintigraphy is a test that shows the areas of rapid growth and bone remodeling. Since this is the whole skeleton scintigraphy, the search shows the possible involvement of other bone tumors.
The last step in the diagnostic evaluation of patients with bone tumor is biopsy. A biopsy involves taking a tissue sample for histological analysis. It can be done with a needle (needle biopsy or aspiration biopsy is reserved for softer lesions) or through a small incision (incisional biopsy).
TREATMENT OF BONE TUMORS
Based on the clinical findings, results of diagnostic tests and histological analysis of tumor biopsy three very important factors are examined: the type of tumor, anatomic accommodation and presence of metastases, and the optimal treatment plan. There are three main therapeutic modalities: surgery, chemotherapy and radiation.
In the treatment of children and adolescents with malignant bone tumors most commonly is used combination of chemotherapy, surgery and radiation. Treatment conducted by a team of specialists is led by a pediatric oncologist. While orthopedic surgery removes the tumor, with chemotherapy is achieved preoperative degradation of the tumor (necrosis) and with that can be controlled microscopic distant metastases.
Chemotherapy means the treatment of cancer with chemicals called cytostatics. Cytostatics destroy cancer cells by different acting on cell division, so for better antitumor effect it is best to simultaneously apply more medication - polychemotherapy. The value of chemotherapy may be after proved after surgery objectively, by observing the percentage of necrotic tumor cells. A higher percentage of necrosis indicates greater effectiveness of chemotherapy. The first level is tumor necrosis less than 50%, which is a poor response to treatment, necrosis of 50% to 90% is the second level, more than 90% necrosis of the third level, the fourth level is 100% necrosis, indicating the best answer to chemotherapy.
Surgical treatment of bone tumors was significantly changed in the past quarter century. Rescue surgery involves removal of certain tumors in the block with the surrounding healthy tissue, while preserving the limb. Amputations were for many years a standard in the treatment of malignant tumors. Although it is rarely performed today, it still has an important place in the surgical treatment of primary bone tumors. Amputation may be conducted through the tumor (subtotal amputation), through the reactive zone (peripheral amputation) through healthy surrounding bone (wide amputation) or by removing the entire affected bone (radical amputation). Local surgical treatment of benign tumors may be cleaning formations (called curettage) or removal of the entire formation (resection).