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Bone Tumors Benign Ppt


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Bone Tumors Benign Ppt

  2. 2. INITIAL EVALUATION <ul><li>Carried out in 4 phases – </li></ul><ul><li>1 st phase – involves </li></ul><ul><li>High index of suspicion for tumors </li></ul><ul><li>Routine X-rays </li></ul><ul><li>Routine lab facilities </li></ul><ul><li>Meticulous history </li></ul><ul><li>Thorough physical examination </li></ul><ul><li>2 nd phase – is prebiopsy regional evaluatn, to determine size,location and type of tissue in tum. </li></ul><ul><li>3 rd phase – is the actual biopsy. </li></ul><ul><li>4 th phase – is undertaken if presumptive clinical & path evidence sugestive of malignancy, search for mets is done, using CT scan of lung & Tc-99 bone scan. </li></ul>
  3. 3. PRESENTING SYMPTOMS <ul><li>Pain </li></ul><ul><li>Mass </li></ul><ul><li>Patient may present with </li></ul><ul><li>An abnormal radiographic finding detected during evaluation of unrelated problem </li></ul><ul><li>PAIN:- is most frequent symptom </li></ul><ul><li>-deep constant pain,poorly localised,worse at night </li></ul><ul><li>-initially controlled by analgesics,later requires narcotics </li></ul><ul><li>MASS:- rate of enlargement is important </li></ul><ul><li>-Fluctuating mass can be cyst,ganglion or hemangioma </li></ul><ul><li>-Family H/O masses near the joint may be indicator of Ollier’s disease or Maffucci Syndrome </li></ul><ul><li>NEUROLOGICAL SYMPTOM:- found in few patients such as sacral tumors & with tumors located near the nerve causing compression of nerve,especially common in sciatic notch ,inguinal canal & popliteal fossa </li></ul><ul><li>UNEXPLAINED SWELLING OF THE LOWER EXTREMITY :- found in pelvic </li></ul><ul><li>tumors which are painless & without a palpable mass & cause swelling due to compression of iliac vein. </li></ul>
  4. 4. HISTORY OF THE PATIENT <ul><li>AGE:- m/b most imp information,bcoz of their presentaion in sp age group. </li></ul><ul><li>1 st decade- usually ABC ,SBC </li></ul><ul><li>2 nd decade-Chondroblastoma,osteosarcoma,Ewings </li></ul><ul><li>3 rd decade- GCT </li></ul><ul><li>4 th decade- chondrosarcoma </li></ul><ul><li>5 th decade- Multiple myeloma </li></ul><ul><li>SEX:- less imp than age </li></ul><ul><li>Some tumors like GCT are more in females </li></ul><ul><li>RACE:- little imp, Ewings rare in african descent </li></ul><ul><li>H/O any exposure to radiation Tt or Carcinogens- bone seeking radionucleotide can cause sarcoma. </li></ul><ul><li>Various chemlcal carcinogens- methylcholanthrene,zinc beryllium silicate, beryllium oxide. </li></ul><ul><li>Currently the most worrisome & controversial is Nickel which is used in many orthopedic devices. </li></ul>
  5. 5. PHYSICAL EXAMINATION <ul><li>Evaluation of patient’s general health </li></ul><ul><li>TUMOR MASS should be measured & its location,shape, consistency,mobility,tenderness,local temp & change with position should be noted. </li></ul><ul><li>SKIN & SUBCUTANEOUS TISSUE : </li></ul><ul><li>Small dialated superficial veins overlying the mass are produced by large tumors </li></ul><ul><li>Café-au-lait spots & subcutaneous neurofibromas indicate Von Recklinghausen’s disease </li></ul><ul><li>A venous malformation on the same of body as the cartilagenous tumor is an indicator of Maffucci Syndrome </li></ul><ul><li>REGIONAL LYMPH NODES: sign of metastatic disease </li></ul><ul><li>Atrophy of surrounding musculature should be recorded,also neurological deficits & adequacy of circulation. </li></ul>
  6. 6. LABORATORY TEST <ul><li>Alkaline phosphatase test: Normally, this enzyme is present in high levels when bone-forming cells are very active (usually in young children when their bones grow or when a broken bone mends). High levels of alkaline phosphatase can also be an indicator of bone tumors (when the tumor creates abnormal bone tissues). </li></ul><ul><li>PTH test: Lower-than-normal level of parathormone can be an indicator of bone cancer. </li></ul><ul><li>Serum phosphorus: Higher than normal levels of phosphorus can be an indicator of bone cancer. </li></ul><ul><li>Ionized calcium and serum calcium: Higher than normal levels of calcium can be an indicator of bone cancer. </li></ul>
  7. 7. OTHER TESTS <ul><li>Hemoglobin </li></ul><ul><li>CBC </li></ul><ul><li>ESR </li></ul><ul><li>CRP </li></ul><ul><li>Glucose tolerance test </li></ul><ul><li>PSA,PAP </li></ul><ul><li>Electrophoresis & urinary Bence Jones protein </li></ul>
  8. 8. INVESTIGATIONS <ul><li>X-RAY </li></ul><ul><li>CT SCAN </li></ul><ul><li>MRI </li></ul><ul><li>TECHNETIUM BONE SCAN-This type of scan uses a very low radioactive material (diphosphonate) to see whether or not the cancer has spread to other bones and the damage suffered by the bone. </li></ul><ul><li>PET- Positron Emission Tomography uses radioactive glucose to locate cancer. This glucose contains a radioactive atom that is absorbed by the cancerous cells and then detected by a special camera. </li></ul>
  9. 9. BIOPSY <ul><li>The biopsy is the most conclusive test because it confirms if the tumor is malignant or benign, the bone cancer type (primary or secondary bone cancer), and stage. </li></ul><ul><li>According to the tumor size and type (malignant or benign) and the biopsy's purpose (to remove the entire tumor or only a small tissue sample), there are two types of biopsies used in bone cancer diagnosis. These are: needle biopsy and incisional biopsy. </li></ul><ul><li>1. Needle biopsy: During this procedure, a small hole is made in the affected bone and a tissue sample from the tumor is removed. </li></ul><ul><li>There are two types of needle biopsies: </li></ul><ul><li>Fine needle aspiration : During this procedure, the tissue sample is removed with a thin needle attached to a syringe. </li></ul><ul><li>Core needle aspiration : During this procedure, the surgeon removes a small cylinder of tissue sample from the tumor with a rotating knife like device. </li></ul><ul><li>2. Incisional biopsy : During this procedure, the surgeon cuts into the tumor and removes a tissue sample. </li></ul>
  10. 11. STAGING <ul><li>Enneking described the most widely used staging system for benign bone tumors . </li></ul><ul><li>The stages are denoted by the Arabic numerals 1, 2, and 3, whereas malignant bone tumors are classified by Roman numerals (I, II, III). </li></ul><ul><li>Many benign bone tumors have the potential to present at, and progress through, various stages during their disease course. </li></ul><ul><li>Stage 1-LATENT, it do not have any characteristics of growth or progressive change, may resolve spontaneously. </li></ul><ul><li>Stage 2-ACTIVE , lesion deform the host bone but remain contained in bone, require intralesional curettage. </li></ul><ul><li>Stage 3-AGGRESSIVE , tumor extend beyond the bone, require complete work-up and a removal with wide margins to avoid possible local recurrence. </li></ul>
  11. 12. <ul><li>The staging system for malignant tumors adopted by the Musculoskeletal Tumor Society, and originally developed by Enneking is based on the histological grade, the local extent, and the presence or absence of metastasis. </li></ul><ul><li>Stage Grade Local Extent Metastasis </li></ul><ul><li>1A low intracompartmental - </li></ul><ul><li>1B low extracompartmental - </li></ul><ul><li>2A high intracompartmental - </li></ul><ul><li>2B high extracompartmental - </li></ul><ul><li>3 any any + </li></ul><ul><li>Grade is determined by histological parameters. Low-grade tumors generally have few mitotic figures, little if any cellular atypia, and have a relatively non-infiltrative growth pattern. High-grade tumors tend to have marked cellular atypia, hyperchromatism, and nuclear pleomorphism. They often demonstrate an infiltrative growth pattern </li></ul>
  12. 14. OSTEOCHONDROMA <ul><li>Osteochondromas occur as solitary osteochondroma (osteocartilaginous exostosis) or as multiple osteochondromatosis. </li></ul><ul><li>Solitary Osteochondroma is a developmental abnormality of bone. It occurs when part of the growth plate forms an outgrowth on the surface of the bone. This bone outgrowth may or may not have a stalk. When a stalk is present, the structure is called pedunculated. When no stalk is present, it is called sessile. </li></ul><ul><li>An osteochondroma may grow in a child or adolescent. Its growth usually stops at maturity. </li></ul><ul><li>Solitary osteochondromas are thought to be the most common benign bone tumor. </li></ul><ul><li>It is seen in patients aged 10 to 30. It occurs equally in males and females. It does not result from injury. It is thought to arise during skeletal growth when bone grows away from the growth plate instead of in line with it. Cause of solitary osteochondroma is unknown. </li></ul>
  13. 15. SYMPTOMS <ul><li>The most common symptom of an osteochondroma is a painless mass near the joints. The knee and shoulder are more commonly involved. It can be found at the ends of any long bone and along the pelvis. </li></ul><ul><li>If the stalk of a pedunculated osteochondroma breaks, pain and swelling may start immediately. </li></ul><ul><li>It can be located under a tendon. Snapping of the tissue over the tumor may cause activity-related pain. </li></ul><ul><li>It can be located near a nerve or blood vessel, such as behind the knee causing numbness and tingling in that extremity, periodic changes in blood flow, loss of pulse or changes in color of the limb. </li></ul>
  14. 16. DIAGNOSIS <ul><li>A plain X-ray will show the bony growth. </li></ul><ul><li>MRI scan may be used to look for cartilage on the surface of the bony growth. Such cartilage in an adult patient should be checked for cancer if it is >2 cm in size, or if there is pain. </li></ul><ul><li>CT scan may also be used. </li></ul>
  15. 17. TREATMENT <ul><li>Nonsurgical Treatment </li></ul><ul><li>Most of the time, solitary osteochondroma is not removed surgically. Observe it & take regular X-rays to keep track of any changes. </li></ul><ul><li>Surgical Treatment </li></ul><ul><li>When surgery is recommended, it is best to wait until growth complete (a mature skeleton by X-ray evaluation) before removing a solitary osteochondroma. This decreases the chance of the tumor growing back. </li></ul><ul><li>Surgery may be considered if the osteochondroma: </li></ul><ul><li>Is causing pain with activity </li></ul><ul><li>Puts pressure on a nerve or blood vessel </li></ul><ul><li>Has a large cap of cartilage </li></ul><ul><li>The osteochondroma is removed at the level of the normal bone. Some of the inside of the bone may also be removed. </li></ul><ul><li>Osteochondromas are thought to be associated with a gene called EXT 1. </li></ul>
  16. 18. MULTIPLE OSTEOCHONDROMATOSIS <ul><li>Multiple osteochondromatosis is also called multiple osteocartilaginous exostosis, multiple hereditary exostosis (MHE), familial osteochondromatosis, multiple hereditary osteochondromatosis, or Diaphyseal aclasia. </li></ul><ul><li>About 70 percent of the time, it is inherited. About 30 percent of the time, it occurs randomly. </li></ul><ul><li>It affects males more often than females. It usually happens within the first three decades of life. </li></ul><ul><li>Alterations in genes called EXT genes are thought to be the cause of this disease. It can be passed along in families. There is no other known risk factor or cause. There is increased risk of malignant transformation. </li></ul>
  17. 19. SYMPTOMS <ul><li>Symptoms are the same as solitary osteochondroma. The number and location of osteochondromas varies. A patient may have deformities of the forearms, a short stature, and knock-knees and ankles. This is caused by abnormal growth in the bones affected by the osteochondromas. </li></ul><ul><li>Symptoms and signs of a tumor becoming cancerous are: </li></ul><ul><li>Growth of an osteochondroma after puberty </li></ul><ul><li>Pain at the site of an osteochondroma </li></ul><ul><li>A cartilage cap >2cm </li></ul>
  18. 20. DIAGNOSIS <ul><li>It is diagnosed when two or more osteochondromas are seen on plain X-rays. Multiple osteochondromatosis has been associated with EXT 1 and EXT 2 genes. </li></ul><ul><li>X-ray findings are the same as with solitary osteochondromas. </li></ul><ul><li>If cancer is suspected, you may need a thorough evaluation. This includes MRI & CT scans of the tumor. </li></ul><ul><li>Pain at the site of a mass or growth after puberty should be evaluated. The most common malignancy found in this transformation is called chondrosarcoma. </li></ul><ul><li>CT scan of the chest helps look for metastasis to the lungs. </li></ul><ul><li>Biopsy can also be done </li></ul>
  19. 21. TREATMENT <ul><li>Nonsurgical Treatment </li></ul><ul><li>Most of the time, multiple osteochondromas are not removed surgically. Tumors that do not show signs of cancer are watched carefully. Deformities such as knock-knees or ankles may need surgery to straighten the bone. </li></ul><ul><li>Surgical Treatment </li></ul><ul><li>The tumor may be completely removed. This may also include taking out a whole portion of bone, which may need to be replaced with a prosthesis. </li></ul><ul><li>Chemotherapy and radiation therapy are not usually needed. </li></ul>
  20. 22. UNICAMERAL BONE CYST <ul><li>A unicameral (simple) bone cyst is a cavity found within a bone that is filled with straw-colored fluid. It is a benign condition. </li></ul><ul><li>UBC are usually found in patients younger than 20 years of age.It occurs in one bone, in one location. The location of the cysts tends to be in proximal humerus or proximal femur. Less common locations include the pelvis, ankle (talus), or heel (calcaneus). </li></ul><ul><li>There is no known cause. There are theories involving a growth defect or disturbance at the epiphyseal plate leading to the formation of a unicameral bone cyst. </li></ul>
  21. 23. SYMPTOMS <ul><li>Most unicameral bone cysts have no symptoms. Some are discovered incidentally. Other unicameral bone cysts are not noticed at all. </li></ul><ul><li>If a unicameral bone cyst is thinning the bone, there may be pain with weightbearing activities. </li></ul><ul><li>If there is a pathologic fracture through the cyst, the affected arm or leg may have pain, swelling, and deformity. </li></ul>
  22. 24. DIAGNOSIS <ul><li>plain X-rays to show a hollow cavity in the metaphyseal portion of the bone. </li></ul><ul><li>There is no associated bone expansion. A well-defined cortical rim can be seen. On rare occasions, the cyst may cross the physis of an immature patient and extend into the epiphysis. </li></ul><ul><li>If the UBC is not typical in its appearance,CT & MRI may help determine the architecture of the bone. </li></ul>
  23. 25. TREATMENT <ul><li>Nonsurgical Treatment </li></ul><ul><li>A unicameral bone cyst without pain or other symptoms can be watched with repeated X-rays </li></ul><ul><li>Surgical Treatment </li></ul><ul><li>Only unicameral bone cysts at risk of a pathologic fracture need treatment. </li></ul><ul><li>If the unicameral bone cyst is painful, growing larger, or in a location that may fracture, may be treated by aspiration and injecting it with a steroid or with demineralized bone from the bone bank, supplemented occasionally with bone marrow from the patient's pelvis. This method of treatment may require more than one aspiration/injection. </li></ul><ul><li>A unicameral bone cyst in a structurally compromising location may need surgery including scraping the inside of the bone (curettage). The hole left following this may require donor bone or a bone graft substitute to fill the defect. </li></ul><ul><li>Pathologic fractures occasionally incite a healing process. Depending on the size and location of the cyst, treatment with an internal fixation device may also be needed. </li></ul>
  24. 26. OSTEOID OSTEOMA <ul><li>    Osteoid Osteoma is a benign bone lesion with a nidus of <2 cm surrounded by a zone of reactive bone. The tumor occurs most frequently in the second decade and affects males twice as often as females. </li></ul><ul><li>The proximal femur is the most common location followed by the tibia, posterior elements of the spine, and the humerus.It is found in the diaphysis or the metaphysis of the proximal end of the bone. </li></ul><ul><li>SYMPTOMS :- </li></ul><ul><li>It has a distinct clinical picture of dull pain that is worse at night and disappears within 20 to 30 minutes of treatment with non-steroidal anti-inflammatory medication. </li></ul><ul><li>Joint pain may be present with a periarticular lesion and synovitis can occur secondary to an intraarticular lesion. Local symptoms can include an increase in skin temperature, increased sweating and tenderness. Epiphyseal lesions can cause abnormal growth. </li></ul>
  25. 27. DIAGNOSIS <ul><li>The classic radiological presentation of an osteoid osteoma is a radiolucent nidus surrounded by a dramatic reactive sclerosis in the cortex of the bone. The center can range from partially mineralized to osteolytic to entirely calcified. The lesion can occur only in the cortex, in both the cortex and medulla, or only the medulla. The reactive sclerosis may be present or absent. The four diagnostic features include </li></ul><ul><li>(1) a sharp round or oval lesion that is </li></ul><ul><li>(2) less than 2 cm in diameter, </li></ul><ul><li>(3) has a homogeneous dense center and </li></ul><ul><li>(4) a 1-2 mm peripheral radiolucent zone.' </li></ul><ul><li>  CT is the preferred method of evaluation, especially if the lesion is in the spine or obscured by reactive sclerosis. The radiologic D/D includes osteoblastoma, osteomyelitis, arthritis, stress fracture and enostosis </li></ul>
  26. 28. TREATMENT <ul><li>Osteoid osteoma will resolve without treatment in an average of 33 months. </li></ul><ul><li>If the patient does not wish to endure the pain and prolonged use of non-steroidal anti-inflammatory medications, surgical removal or percutaneous ablation of the nucleus is indicated. </li></ul>
  27. 29. CHONDROBLASTOMA <ul><li>Sometimes called Codman's tumor , a chondroblastoma is a rare type of benign bone tumor that originates from cartilage. Chondroblastoma most often affects the ends of the long bones in the arms and legs at the hip, shoulder, and knee. </li></ul><ul><li>Chondroblastoma is a rare bone tumor that can affect people of all ages, most common between the ages of 10 and 20 years. This type of tumor is also more common in males than females. </li></ul><ul><li>The exact cause of chondroblastoma is not known. The tumors are believed to originate from immature cartilage producing cells called chondroblasts. </li></ul>
  28. 30. SYMPTOMS <ul><li>Symptoms of chondroblastoma may vary depending on the location of the tumor. However, each individual may experience symptoms differently. Symptoms may include: </li></ul><ul><li>pain in the knee, hip, and shoulder joint. </li></ul><ul><li>withered or shrunken appearance of the muscle near the affected bone </li></ul><ul><li>impaired mobility of the adjacent joint </li></ul><ul><li>fluid accumulation in the joint adjacent to the affected bone </li></ul>
  29. 31. DIAGNOSIS <ul><li>X-RAY : The diagnosis of chondroblastoma can usually be made by radiograph.The most common site for chondroblastoma is the epiphysis. The lesion is lytic with well defined margins and can be from 1-6cm in size. Scalloping or expansion of cortical bone may be present. Fine calcifications, either punctate or in rings, may be visible. </li></ul><ul><li>MRI & CT : Cysts are present about 20% of the time and both MRI and CT can define the fluid levels. CT is also useful for defining the relationship of the tumor to the joint, integrity of the cortex, and intralesional calcifications. </li></ul><ul><li>The differential diagnosis includes enchondroma, central chondrosarcoma and aneurysmal bone cyst. </li></ul>
  30. 32. <ul><li>Chondroblastoma is made up of uniform, polygonal cells that are closely packed. These primitive cells are derived from the epiphyseal cartilage plate and have abundant cytoplasm. There is little mitotic activity. A scant chondroid matrix may be superimposed by a pericellular deposit of calcification that appears like &quot; chicken-wire &quot;. Giant cells are often present. </li></ul>
  31. 33. TREATMENT <ul><li>The goal for treatment of chondroblastoma is to remove the tumor and prevent damage to the end of the affected bone. </li></ul><ul><li>Treatment may include: </li></ul><ul><li>Surgical removal of the tumor - Biopsy and curettage with possible use of  adjuvant liquid nitrogen or phenol, or a mechanical burr. It may be necessary to reconstruct articular surfaces due to subchondral erosion. </li></ul><ul><li>Any joint invasion is usually secondary to previous instrumentation. All pulmonary nodules should be excised. </li></ul><ul><li>Bone grafting - a surgical procedure in which healthy bone is transplanted from another part of the patient's body into the affected area, if necessary, to repair damaged bone. </li></ul><ul><li>Physical therapy (to restore strength and function after surgery) </li></ul>
  32. 34. CHORDOMA <ul><li>Chordoma is a rare tumor that usually occurs in the spine and base of the skull. </li></ul><ul><li>It is a malignant tumor that grows fairly slowly. It can spread to other organs, usually the lungs. It represents only about 1 percent of all malignant bone tumors. </li></ul><ul><li>Chordomas develop in a structure called the notochord. The notochord forms the early spine in the beginning stages of fetal development. Most of the notochord is replaced by the spine during the first six months of development. Small areas can remain, and chordomas can form in these areas. </li></ul><ul><li>Most chordomas occur at the base of the spine (sacrum), in the tailbone (coccyx) or at the base of the skull (40 percent). </li></ul><ul><li>between 40 and 70 years of age, Occasionally in younger patients, even children. </li></ul><ul><li>Chordomas are life-threatening. They can cause death by direct growth or by spreading to other organs. They spread to the lungs about 20 to 30 percent of the time. </li></ul>
  33. 35. RISK FACTORS <ul><ul><ul><ul><ul><li>Chordomas occur spontaneously. </li></ul></ul></ul></ul></ul><ul><li>They are not known to be caused by trauma, environmental factors, or diet. </li></ul><ul><li>Chordomas are not inherited,not associated with any medical conditions or use of any medications or supplements. </li></ul>
  34. 36. SYMPTOMS <ul><li>Symptoms depend on the location of the tumor. </li></ul><ul><li>Tumors in the skull can cause headache or problems with vision. </li></ul><ul><li>Tumors in the spine can cause pain in the area of tumor (neck, back, or tailbone). A tumor pressing on nearby nerves can cause symptoms similar to a &quot;slipped&quot; (herniated) disk. These symptoms include arm or leg pain, weakness, or numbness. </li></ul><ul><li>Tumors in the tailbone area can cause a noticeable mass, numbness in the crotch area, and problems with the bowel and bladder. </li></ul>
  35. 37. DIAGNOSIS <ul><li>X-rays of the sacrum are often difficult to interpret. </li></ul><ul><li>The plain radiographic hallmark of a chordoma is midline bony destruction with a large associated soft-tissue mass </li></ul><ul><li>In the vertebral body, the chordoma is lytic, centrally located, and slowly expansile. Areas of sclerosis due to reactive bone formation are seen. Adjacent vertebral bodies and the intervening disc space can be involved. </li></ul><ul><li>Reports vary as to the activity of a chordoma on bone scan, and accumulation of isotope in the bladder can obscure the sacral area. </li></ul><ul><li>CT scans and MRI - extremely helpful in determining the extent of the lesion and its proximity to vital structures. This is essential in preoperative planning. CT scans identify calcified areas that are not evident on plain films. Along with myelography, CT is helpful in planning resection of a vertebral lesion. </li></ul><ul><li>MRI is useful in discovering recurrent nodules after surgical resection. </li></ul><ul><li>Angiography is only occasionally indicated to identify the proximity of a cervical chordoma to the vertebral arteries. </li></ul><ul><li>The radiographic differential diagnosis includes metastatic disease, multiple myeloma, giant cell tumor, and neurogenic tumors. </li></ul>
  36. 38. TREATMENT <ul><li>Treatment of chordomas is very difficult. This is mainly because these tumors are near the brain and spinal cord. </li></ul><ul><li>Traditional chemotherapy has not been very effective. </li></ul><ul><li>Surgery is preferred, where possible. Radiation in combination with surgery is often used to treat these tumors. Radiation alone rarely cures the tumor. When tumors cannot be surgically removed, sometimes radiation therapy can stop the growth of the tumor. </li></ul><ul><li>The surgeon must remove the tumor and a margin of normal tissue around it. This often results in a loss of neurologic function. For example, surgical removal of tumors in the sacrum can result in loss of control of bowel and bladder function (incontinence). </li></ul><ul><li>New drug treatments, including use of a drug called imatinib mseylate are being investigated which may help slow the growth of tumors that can't be removed. </li></ul>
  37. 39. ENCHONDROMA <ul><li>Enchondroma is one type of benign cartilage tumor that appears on the inside of the bone. These tumors usually begin and grow in childhood, then stop growing but remain present throughout adulthood. They are often found in patients between 10 and 20 years of age. Some cases become dormant or burned out. </li></ul><ul><li>very common in the small bones of the hand and feet. In fact, they are the most common tumor of the hand. They also occur in the long bones of the upper arm and thigh. </li></ul><ul><li>In rare cases, multiple tumors can appear as part of a syndrome. These syndromes are Ollier's disease and Maffucci's syndrome. Single enchondromas can become cancerous, but this is very rare. The rate of change to a cancerous tumor is a little higher in Ollier's disease and Maffucci's syndrome. </li></ul><ul><li>The cause of enchondromas is not completely clear, but they don't seem to be caused by exposure to chemicals or radiation or patient activities. </li></ul><ul><li>The chance that a single one of these tumors will become malignant is very low. For the very few that become cancer, when they become malignant, they usually become a chondrosarcoma. </li></ul>
  38. 40. SYMPTOMS <ul><li>Enchondromas are usually painless,found during an X-ray evaluation of another problem in the same area. When these tumors appear in the hands or feet, or in multiple lesions, they can deform the bone, lead to pathologic fracture. </li></ul><ul><li>The deformities in patients with Ollier's disease and Maffucci's syndrome can be quite severe. </li></ul><ul><li>If pain from other nearby sources has been excluded, the tumor should be carefully studied to determine whether it could be a low-grade chondrosarcoma. Pain at night or at rest is more likely a malignant tumor but pain is also very common with some shoulder conditions like rotator cuff tendonitis. </li></ul>
  39. 41. Diagnosis <ul><li>Most enchondromas are found when X-rays are taken for another reason, such as injury or arthritis. The diagnosis of an enchondroma is important to make sure it is not a more aggressive or cancerous tumor. </li></ul><ul><li>It is important to know if the tumor is painful and the kind of pain it causes. There is greater concern if the pain occurs at rest or if the pain occurs at night and does not go away. Pain caused by activity is not as troublesome. Often pain from a different reason in a nearby joint may have prompted the X-ray. </li></ul><ul><li>on the X-rays. Enchondromas appear as small (less than 5 cm) lobe-shaped white tumors in the middle of the bone. The white areas of the tumor usually show a pattern of rings and arcs that indicates the tumor contains cartilage. </li></ul><ul><li>Characteristics of a more aggressive tumor or malignant chondrosarcoma include: </li></ul><ul><li>Scalloping of the inner surface of the bone </li></ul><ul><li>Thickening of the bone cortex </li></ul><ul><li>Destruction of the bone by the tumor </li></ul><ul><li>Soft-tissue mass </li></ul><ul><li>Bone erosion that is growing </li></ul><ul><li>Erosion surrounded by reactive bone </li></ul><ul><li>Most enchondromas are diagnosed on plain X-rays. </li></ul>
  40. 42. <ul><li>CT or MRI scan give a more complete picture of the bone around the tumor. . </li></ul><ul><li>Bone scans - both benign and malignant tumors can show increased uptake of the radioactive tracer used to detect increased bone activity. </li></ul><ul><li>Under the microscope, enchondromas have islands of cartilage that surrounds them. Usually, cartilage is not found in the center of bones. However, these tumors in the hand and foot or in Ollier's disease or Maffucci's syndrome may contain more odd-looking cartilage. </li></ul><ul><li>Low-grade chondrosarcomas (cancers) look more cellular under a microscope than enchondromas and there is less normal bone in the tumor. </li></ul>A computed tomography (CT) or magnetic resonance imaging (MRI) scan of the affected bone may sometimes be helpful. These scans give a more complete picture of the bone around the tumor. . Bone scans can be helpful in some circumstances. However, both benign and malignant tumors can show increased uptake of the radioactive tracer used to detect increased bone activity. Under the microscope, enchondromas have islands of cartilage that are easy to tell apart from the normal bone that surrounds them. Usually, cartilage is not found in the center of bones. However, these tumors in the hand and foot or in Ollier's disease or Maffucci's syndrome may contain more odd-looking cartilage. They may be more difficult to tell apart from a mildly cancerous malignant tumor called low-grade chondrosarcoma. Low-grade chondrosarcomas (cancers) look more cellular under a microscope than enchondromas and there is less normal bone in the tumor. Because both low-grade chondrosarcomas and enchondromas look similar under the microscope, a group effort between experienced surgeons, radiologists, and pathologists is important to get the best interpretation of the tumor.
  41. 43. TREATMENT <ul><li>Nonsurgical Treatment </li></ul><ul><li>Most enchondromas require no treatment at all. When needed, treatment for enchondroma can vary due to the difficulty in determining the aggressiveness of the lesion. </li></ul><ul><li>Stage 1 latent lesions - that show no symptoms can be watched with regular X-rays to ensure the tumor is not growing. Most surgeons think that tumors without symptoms do not need to be removed. </li></ul>
  42. 44. Surgical Treatment <ul><li>Sx Tt is usually with scraping out and filling of the cavity with bone graft or other filling substances. Although they can come back (recur), most of them will not. </li></ul><ul><li>Tumors that cause pathologic fractures are usually treated by allowing the fracture to heal. Then, the tumor is scraped out to prevent another fracture. </li></ul><ul><li>More aggressive tumors with bone destruction or with a mass growing outside the bone are usually chondrosarcomas. These tumors need to be removed. </li></ul><ul><li>Malignant tumors are either scraped out or the entire bone around the lesion must be removed. This decision is made depending on the grade of the tumor. The grade of the tumor is determined by imaging studies and biopsy. </li></ul><ul><li>Unfortunately, a biopsy will not often help in these cases. Even specialized bone pathologists can have trouble determining the difference between a benign enchondroma and a low grade chondrosarcoma. </li></ul>
  43. 45. GIANT CELL TUMOR <ul><li>Giant cell tumor of bone is a rare, aggressive non-cancerous (benign) tumor. </li></ul><ul><li>occurs in adults between the ages of 20 and 40 years. very rarely seen in children or in adults older than 65 years of age. Giant cell tumors occur in approximately one person per million per year. </li></ul><ul><li>Giant cell tumors are named for the way they look under the microscope. Many &quot;giant cells&quot; are seen. They are formed by fusion of several individual cells into a single, larger complex. </li></ul><ul><li>Many bone tumors and other conditions (including normal bone) contain giant cells. Giant cell tumor of bone is given its characteristic appearance by the constant finding of a large number of these cells existing in a typical background. </li></ul><ul><li>Giant cell tumor of bone occurs almost exclusively in the end portion of long bones next to the joints (epiphysis). In rare cases, this tumor may spread to the lungs. </li></ul><ul><li>Giant cell tumors of bone occur spontaneously. They are not known to be associated with trauma, environmental factors, or diet. They are not inherited. </li></ul><ul><li>In rare cases, they may be associated with hyperparathyroidism. </li></ul>
  44. 46. Giant Cell Tumor Osteoclastoma of Bone in Rib &quot;Soap Bubble&quot; appearance on X-ray
  45. 47. SYMPTOMS <ul><li>GCT most frequently occur around the knee joint in the lower end of the femur or the upper end of the tibia. Other common locations include the wrist (lower end of the radius), the hip (upper end of the femur), the shoulder (upper end of the humerus), and lower back (connection of the spine and pelvis). </li></ul><ul><li>The bone involved is generally tender. There may be swelling in the area around it. </li></ul><ul><li>The first symptom is pain in the area of the tumor. There may be pain with movement of a nearby joint. Pain generally increases with activity and decreases with rest. Pain is usually mild initially, but it progressively increases. Occasionally the bone weakened by the tumor may break and cause sudden onset of severe pain. </li></ul>
  46. 48. DIAGNOSIS <ul><li>Giant cell tumors appear on X-rays as destructive (lytic) lesions next to a joint. Occasionally, the involved area of bone can be surrounded by a thin rim of white bone that may be complete or incomplete. The bone in the area of the tumor is sometimes expanded. </li></ul><ul><li>MRI and CT scans better evaluate the area of involvement. </li></ul><ul><li>bone scan generally shows a &quot;hot spot&quot; in the bone where the tumor is. An X-ray or CT scan of the chest will often be done to look for possible spread to the lungs </li></ul>
  47. 49. Treatment <ul><li>If not treated, these tumors will continue to grow and destroy bone. Surgery has proven to be the most effective treatment for giant cell tumors. </li></ul><ul><li>Nonsurgical Treatment </li></ul><ul><li>Radiation therapy is a nonsurgical effective treatment option. However, this can result in the formation of cancer in as many as 15 percent of patients who receive it. Therefore, radiation therapy is used only in the most difficult cases where surgery cannot be performed safely or effectively. </li></ul><ul><li>Some centers have reported successful treatment of giant cell tumors with Embolization- a process where the blood vessels supplying the tumor are occluded by means of a catheter threaded through the main arteries. Blocking the blood supply to the tumor can cause it to shrink and even disappear because the tumor loses its source of nutrition. </li></ul><ul><li>Clinical trials of Drug interferon are currently underway. works by interfering with the ability of the tumor to grow new blood vessels. Because tumors require an ever-increasing blood supply to grow, interferon may also cause the tumor to shrink or even disappear. </li></ul><ul><li>Like radiation therapy,, embolization and treatment with interferon are generally reserved for those tumors that are difficult to remove surgically or in situations where the tumor keeps returning despite treatment or if it spreads. </li></ul>
  48. 50. Surgical Treatment <ul><li>If treatment is limited to curettage and bone grafting alone, the tumor may come back (recur) up to 45 percent of the time. The use of bone cement rather than bone graft results in a lower rate of recurrence. </li></ul><ul><li>Scooping out the tumor (curettage) is generally performed. This techniques cause a hole in the bone that can be filled with a bone graft from other parts of the patient's own body (autograft) or from a cadaver (allograft). </li></ul><ul><li>Enhancing the curettage with a high-speed burr or with the use of agents such as liquid nitrogen, hydrogen peroxide, or phenol, followed by placement of bone cement decreases the recurrence rate to 10 percent to 29 percent. </li></ul><ul><li>More complex tumor removal and reconstruction is sometimes necessary in situations where the tumor has caused excessive damage or recurred. </li></ul><ul><li>In the rare cases where the tumor has spread to the lungs, surgical removal of the tumor in the lungs results in cure 75 percent of the time. </li></ul>
  49. 51. MULTIPLE MYELOMA/ PLASMACYTOMA <ul><li>Causes </li></ul><ul><li>MM may occur spontaneously </li></ul><ul><li>On exposure to ionizing radiation and the pesticide dioxin </li></ul><ul><li>Infection with some viruses (HIV and human herpes 8) has also been associated with multiple myeloma. </li></ul><ul><li>No known risk factors are inherited. </li></ul>
  50. 52. SYMPTOMS <ul><li>usually bone pain is main complain. Other symptoms include: </li></ul><ul><li>Fatigue </li></ul><ul><li>Feeling ill </li></ul><ul><li>Fever </li></ul><ul><li>Night sweats </li></ul><ul><li>Weight loss is not common in the early stages. </li></ul><ul><li>patients are pale with diffuse bone tenderness, especially around the sternum (breastbone) and pelvis (hips). </li></ul><ul><li>spine is the most common location for a pathological fracture. It can also happen in the ribs and pelvis. </li></ul><ul><li>Compression of the spinal cord in 10%-15%This causes pain in the back and legs and numbness and weakness in the legs. </li></ul><ul><li>Patients who have high levels of calcium in the blood may experience nausea, fatigue, confusion, constipation, and frequent urination. </li></ul><ul><li>Patients with anemia may experience fatigue, weakness, and shortness of breath with exercise. </li></ul><ul><li>In advanced cases, patients typically have recurrent infections and can have kidney failure. </li></ul>
  51. 53. DIAGNOSIS <ul><li>In addition to a complete medical history and physical examination, diagnostic procedures for myeloma bone disease may include- </li></ul><ul><li>x-ray - a diagnostic test </li></ul><ul><li>When only one lesion is found </li></ul><ul><li>it is called a plasmacytoma, </li></ul><ul><li>Most doctors believe plasmacytoma </li></ul><ul><li>is simply an early, isolated form of </li></ul><ul><li>multiple myeloma. </li></ul><ul><li>blood and urine tests </li></ul><ul><li>bone marrow aspiration and/or biopsy - a procedure that involves taking a small amount of bone marrow fluid (aspiration) and/or solid bone marrow tissue (called a core biopsy), usually from the hip bones, to be examined for the number, size, and maturity of blood cells and/or abnormal cells. </li></ul><ul><li>magnetic resonance imaging (MRI) - a diagnostic procedure. </li></ul>
  52. 54. CHEMOTHERAPY <ul><li>The standard treatment medications are melphalan and prednisone. </li></ul><ul><li>The median survival rate is three years with this treatment alone. For patients in whom this therapy is ineffective, alternatives include: </li></ul><ul><li>VBMCP (vincristine, carmustine, melphalan, cyclophosphamide and prednisone) </li></ul><ul><li>VAD (vincristine, adriamycin and dexamethasone) </li></ul>
  53. 55. <ul><li>a recent advancement in the treatment of multiple myeloma has increased, response rates and survival. </li></ul><ul><li>This treatment consists of high-dose chemotherapy, followed by autologous stem cell transplantation. With this treatment, patients have a 20 percent chance of living longer than 10 years. This stem cell transplantation involves: </li></ul><ul><li>Harvesting a patient's own blood cells </li></ul><ul><li>Conditioning them with very high doses of melphalan </li></ul><ul><li>Re-infusing the blood cells back into the patient </li></ul>
  54. 56. RADIATION THERAPY <ul><li>Radiation therapy is reserved for decreasing the size of symptomatic bone lesions. </li></ul>
  55. 57. SUPPORTIVE CARE <ul><li>Supportive care is critical. Supportive care includes managing the bone disease, anemia, infections, kidney failure, and pain associated with multiple myeloma. </li></ul><ul><li>Bisphosphonates (medication) can prevent destructive bone lesions and spine fractures. </li></ul><ul><li>Erythropoetin or occasional blood transfusions can manage anemia. </li></ul><ul><li>Antibody infusions and vaccinations can help patients with recurrent infections. </li></ul><ul><li>Corticosteroids and hydration can be used to treat high blood calcium concentrations (from bone loss) and dehydration. </li></ul><ul><li>Narcotics can decrease the pain associated with bone lesions. </li></ul><ul><li>Operative intervention may be required to stabilize and control the pain associated with bone fractures. </li></ul>
  56. 58. SURGICAL TREATMENT <ul><li>Surgery will not cure multiple myeloma. Surgery is used to treat fractures and impending fractures in the spine, pelvis, hip, and shoulder. The goal of these surgeries is to decrease pain and maintain function. </li></ul><ul><li>Internal fixation augmented with cement is frequently recommended, as are joint replacements and vertebroplasties (for spine fractures). </li></ul><ul><li>Operative intervention does not alter the survival rate, but it does increase the quality of life. </li></ul>
  57. 59. EWING’S SARCOMA <ul><li>Ewing's sarcoma is a malignant tumor. usually begins growing initially in a bone. Occasionally, it also begins in the soft tissues (extraosseous Ewing's sarcoma). </li></ul><ul><li>Unlike most cancers that are known to arise from a certain kind of tissue or organ for example, osteosarcoma arises from bone cells, and breast carcinoma arises from breast cells. The exact cell of origin for Ewing's sarcoma is not known. </li></ul><ul><li>Recently,the disease has been defined to include a family of tumors. This includes the Ewing's sarcoma of bone or soft tissue, and primitive neuroectodermal tumor (PNET). Sometimes these tumors are called small blue cell (round cell) tumors. They are named for the way they look under a microscope. </li></ul><ul><li>all malignant bone tumors are rare. Among malignant bone tumors in children and young adults, Ewing's sarcoma is the second most common. There is no known cause. </li></ul><ul><li>There are no known risk factors for developing Ewing's sarcoma. There is no known genetic link. </li></ul>
  58. 60. SYMPTOMS <ul><li>There is usually pain and/or swelling at the affected site. A bone weakened by disease may break after a minor injury. </li></ul><ul><li>Ewing's sarcoma usually affects the long bones, such as the femur, tibia, and humerus. The bones of the pelvis may also be affected. </li></ul>
  59. 61. DIAGNOSIS <ul><li>These include X-rays, MRI , CT and bone scans. </li></ul><ul><li>The diagnosis is confirmed by biopsy. This may be done in an operating room or X-ray department. </li></ul><ul><li>Special tests can help to diagnose Ewing's sarcoma. The hallmark is an abnormality in the cell's chromosomes. Genetic material is mismatched. Chromosomes #11 and #22 are affected. </li></ul><ul><li>Blood tests, lung CT scan, bone scan, and bone marrow biopsy are needed to show the extent of disease. This process is known as &quot;staging.&quot; </li></ul><ul><li>Tests can show if cancer has spread to other sites. such as the lungs or bone marrow. </li></ul>
  60. 62. TREATMENT <ul><li>Ewing's sarcoma is usually diagnosed as a primary bone tumor without evidence of spread elsewhere. Even if tests to do not show spread, the treatment strategy assumes that a very small amount of spread (micrometastatic disease) has already happened. </li></ul><ul><li>The main treatments are chemotherapy, surgery, and radiation . Both surgery and radiation are effective treatments for the primary tumor. </li></ul><ul><li>Many surgeons remove the tumor when doing so will not cause a major loss of function of the body part involved. Many doctors use radiation treatment only when a tumor cannot be removed completely or without the patient losing function. </li></ul>
  61. 63. CHEMOTHERAPY <ul><li>Chemotherapy is used to kill the primary tumor and any unknown microscopic spread. </li></ul><ul><li>Chemotherapy uses multiple medications. The most common agents are vincristine , dactinomycin , cyclophosphamide, and doxorubicin . Recent evidence shows the addition of ifosfamide and etoposide helps. </li></ul><ul><li>Chemotherapy is given through an indwelling central venous catheter. This is placed just before treatment is started. </li></ul><ul><li>Chemotherapy is done in cycles. It uses combinations of the various drugs. The time between cycles lets the blood cell count recover. This includes white blood cells and platelets. The drugs depress them. The patient will lose their hair. </li></ul><ul><li>It generally takes one year to finish all the cycles of chemotherapy, with surgery and/or radiation. </li></ul>
  62. 64. SURGERY <ul><li>Surgery is used to remove the primary tumor and occasionally metastatic lesions. </li></ul><ul><li>The main goal of a tumor surgery is to remove the tumor entirely and keep it from returning; the reconstructive issues, while important, are nonetheless secondary goal. </li></ul><ul><li>In many cases, doctors can avoid amputation by surgically removing the primary tumor. Rebuilding the bone and joints is then needed. Infections and problems with prosthesis and wound healing are the most frequent concerns. </li></ul>
  63. 65. RADIATION THERAPY <ul><li>Radiation is used to lower the chance of recurrence. Radiation may be used instead of surgery at sites where surgery is too risky or complicated. </li></ul><ul><li>When radiation treatment is used, daily treatments are given over the course of many weeks.The risks associated with the radiation include: </li></ul><ul><li>Skin damage </li></ul><ul><li>Muscle scarring and loss of joint flexibility </li></ul><ul><li>Damage to nearby organs </li></ul><ul><li>Loss of bone growth in growing children </li></ul><ul><li>Secondary cancers caused by radiation </li></ul>
  64. 66. PROGNOSIS <ul><li>The outcome for patients with Ewing's sarcoma has improved considerably due to improvements in chemotherapy, diagnostic imaging, and reconstructive techniques. </li></ul><ul><li>In general, two thirds of patients without any demonstrable spread will survive at least five years after diagnosis with standard treatment. When tumors come back, it usually happens within the first few years after treatment. </li></ul><ul><li>The absence of known spread is the most important factor in favor of being more likely to survive. Other factors include having excellent response to chemotherapy, primary tumors in the extremities instead of pelvis, and complete removal of the tumor. </li></ul>