BONE TUMORSBONE TUMORSBONE TUMORS

INITIAL EVALUATIONINITIAL EVALUATIONCarried out in 4 phases –Carried out in 4 phases –11stst phase – involves phase – involves

High index of suspicion for tumorsHigh index of suspicion for tumors Routine X-raysRoutine X-rays Routine lab facilitiesRoutine lab facilities Meticulous historyMeticulous history Thorough physical examinationThorough physical examination

22ndnd phase – is prebiopsy regional evaluatn, to determine phase – is prebiopsy regional evaluatn, to determine size,location and type of tissue in tum.size,location and type of tissue in tum.33rdrd phase – is the actual biopsy. phase – is the actual biopsy.44thth phase – is undertaken if presumptive clinical & path phase – is undertaken if presumptive clinical & path evidence sugestive of malignancy, search for mets is done, evidence sugestive of malignancy, search for mets is done, using CT scan of lung & Tc-99 bone scan.using CT scan of lung & Tc-99 bone scan.

PRESENTING SYMPTOMS PRESENTING SYMPTOMS PainPain MassMass

Patient may present withPatient may present with An abnormal radiographic finding detected during evaluation of unrelated problemAn abnormal radiographic finding detected during evaluation of unrelated problemPAIN:-PAIN:- is most frequent symptom is most frequent symptom -deep constant pain,poorly localised,worse at night-deep constant pain,poorly localised,worse at night -initially controlled by analgesics,later requires narcotics -initially controlled by analgesics,later requires narcotics MASS:-MASS:- rate of enlargement is important rate of enlargement is important -Fluctuating mass can be cyst,ganglion or hemangioma-Fluctuating mass can be cyst,ganglion or hemangioma -Family H/O masses near the joint may be indicator of Ollier’s disease or Maffucci -Family H/O masses near the joint may be indicator of Ollier’s disease or Maffucci

SyndromeSyndromeNEUROLOGICAL SYMPTOM:-NEUROLOGICAL SYMPTOM:- found in few patients such as sacral tumors & with found in few patients such as sacral tumors & with

tumors located near the nerve causing compression of nerve,especially common in tumors located near the nerve causing compression of nerve,especially common in sciatic notch ,inguinal canal & popliteal fossasciatic notch ,inguinal canal & popliteal fossa

UNEXPLAINED SWELLING OF THE LOWER EXTREMITYUNEXPLAINED SWELLING OF THE LOWER EXTREMITY:- found in pelvic:- found in pelvic tumors which are painless & without a palpable mass & cause swelling due to tumors which are painless & without a palpable mass & cause swelling due to

compression of iliac vein.compression of iliac vein.

HISTORY OF THE PATIENTHISTORY OF THE PATIENT

AGE:- m/b most imp information,bcoz of their presentaion in sp AGE:- m/b most imp information,bcoz of their presentaion in sp age group.age group.

11stst decade- usually ABC ,SBC decade- usually ABC ,SBC 22ndnd decade-Chondroblastoma,osteosarcoma,Ewings decade-Chondroblastoma,osteosarcoma,Ewings 33rdrd decade- GCT decade- GCT 44thth decade- chondrosarcoma decade- chondrosarcoma 55thth decade- Multiple myeloma decade- Multiple myeloma

SEX:- less imp than ageSEX:- less imp than age Some tumors like GCT are more in femalesSome tumors like GCT are more in females

RACE:- little imp, Ewings rare in african descentRACE:- little imp, Ewings rare in african descentH/O any exposure to radiation Tt or Carcinogens- bone seeking H/O any exposure to radiation Tt or Carcinogens- bone seeking radionucleotide can cause sarcoma.radionucleotide can cause sarcoma.Various chemlcal carcinogens- methylcholanthrene,zinc beryllium Various chemlcal carcinogens- methylcholanthrene,zinc beryllium silicate, beryllium oxide.silicate, beryllium oxide.Currently the most worrisome & controversial is Nickel which is Currently the most worrisome & controversial is Nickel which is used in many orthopedic devices.used in many orthopedic devices.

PHYSICAL EXAMINATIONPHYSICAL EXAMINATIONEvaluation of patient’s general healthEvaluation of patient’s general healthTUMOR MASS should be measured & its location,shape, TUMOR MASS should be measured & its location,shape, consistency,mobility,tenderness,local temp & change with consistency,mobility,tenderness,local temp & change with position should be noted.position should be noted.SKIN & SUBCUTANEOUS TISSUE :SKIN & SUBCUTANEOUS TISSUE :

Small dialated superficial veins overlying the mass are Small dialated superficial veins overlying the mass are produced by large tumorsproduced by large tumors

Café-au-laitCafé-au-lait spots spots & & subcutaneous neurofibromas indicate subcutaneous neurofibromas indicate Von Recklinghausen’s diseaseVon Recklinghausen’s disease

A venous malformation on the same of body as the A venous malformation on the same of body as the cartilagenous tumor is an indicator of Maffucci Syndromecartilagenous tumor is an indicator of Maffucci Syndrome

REGIONAL LYMPH NODES: sign of metastatic diseaseREGIONAL LYMPH NODES: sign of metastatic disease Atrophy of surrounding musculature should be Atrophy of surrounding musculature should be

recorded,also neurological deficits & adequacy of recorded,also neurological deficits & adequacy of circulation.circulation.

LABORATORY TESTLABORATORY TESTAlkaline phosphatase test:Alkaline phosphatase test: Normally, this Normally, this enzyme is present in high levels when bone-enzyme is present in high levels when bone-forming cells are very active (usually in young forming cells are very active (usually in young children when their bones grow or when a broken children when their bones grow or when a broken bone mends). High levels of alkaline phosphatase bone mends). High levels of alkaline phosphatase can also be an indicator of bone tumors (when can also be an indicator of bone tumors (when the tumor creates abnormal bone tissues). the tumor creates abnormal bone tissues). PTH test:PTH test: Lower-than-normal level of Lower-than-normal level of parathormone can be an indicator of bone cancer.parathormone can be an indicator of bone cancer.Serum phosphorus: Serum phosphorus: Higher than normal levels Higher than normal levels of phosphorus can be an indicator of bone of phosphorus can be an indicator of bone cancer.cancer.Ionized calcium and serum calcium: Ionized calcium and serum calcium: Higher Higher than normal levels of calcium can be an indicator than normal levels of calcium can be an indicator of bone cancer. of bone cancer.

OTHER TESTSOTHER TESTS

HemoglobinHemoglobin

CBCCBC

ESRESR

CRPCRP

Glucose tolerance testGlucose tolerance test

PSA,PAPPSA,PAP

Electrophoresis & urinary Bence Jones proteinElectrophoresis & urinary Bence Jones protein

INVESTIGATIONSINVESTIGATIONSX-RAYX-RAYCT SCANCT SCANMRIMRITECHNETIUM BONE SCAN-This type of scan TECHNETIUM BONE SCAN-This type of scan uses a very low radioactive material uses a very low radioactive material (diphosphonate) to see whether or not the (diphosphonate) to see whether or not the cancer has spread to other bones and the cancer has spread to other bones and the damage suffered by the bone. damage suffered by the bone. PET- Positron Emission Tomography uses PET- Positron Emission Tomography uses radioactive glucose to locate cancer. This radioactive glucose to locate cancer. This glucose contains a radioactive atom that is glucose contains a radioactive atom that is absorbed by the cancerous cells and then absorbed by the cancerous cells and then detected by a special camera. detected by a special camera.

BIOPSYBIOPSYThe biopsy is the most conclusive test because it confirms if The biopsy is the most conclusive test because it confirms if the tumor is malignant or benign, the bone cancer type the tumor is malignant or benign, the bone cancer type (primary or secondary bone cancer), and stage. (primary or secondary bone cancer), and stage. According to the tumor size and type (malignant or benign) According to the tumor size and type (malignant or benign) and the biopsy's purpose (to remove the entire tumor or only a and the biopsy's purpose (to remove the entire tumor or only a small tissue sample), there are two types of biopsies used in small tissue sample), there are two types of biopsies used in bone cancer diagnosis. These are: needle biopsy and incisional bone cancer diagnosis. These are: needle biopsy and incisional biopsy.biopsy.1. Needle biopsy:1. Needle biopsy: During this procedure, a small hole is made During this procedure, a small hole is made in the affected bone and a tissue sample from the tumor is in the affected bone and a tissue sample from the tumor is removed. removed. There are two types of needle biopsies:There are two types of needle biopsies:Fine needle aspirationFine needle aspiration: During this procedure, the tissue : During this procedure, the tissue sample is removed with a thin needle attached to a syringe. sample is removed with a thin needle attached to a syringe. Core needle aspirationCore needle aspiration: During this procedure, the surgeon : During this procedure, the surgeon removes a small cylinder of tissue sample from the tumor with removes a small cylinder of tissue sample from the tumor with a rotating knife like device. a rotating knife like device. 2. Incisional biopsy2. Incisional biopsy: During this procedure, the surgeon cuts : During this procedure, the surgeon cuts into the tumor and removes a tissue sample.into the tumor and removes a tissue sample.

STAGINGSTAGINGEnneking Enneking described the most widely used staging system for described the most widely used staging system for

benign bone tumorsbenign bone tumors . . The stages are denoted by the Arabic numerals 1, 2, and 3, The stages are denoted by the Arabic numerals 1, 2, and 3,

whereas malignant bone tumors are classified by Roman whereas malignant bone tumors are classified by Roman numerals (I, II, III). numerals (I, II, III).

Many benign bone tumors have the potential to present at, and Many benign bone tumors have the potential to present at, and progress through, various stages during their disease course.progress through, various stages during their disease course.

Stage 1-LATENT,Stage 1-LATENT, it do not have any characteristics of growth it do not have any characteristics of growth or progressive change, may resolve spontaneously. or progressive change, may resolve spontaneously.

Stage 2-ACTIVEStage 2-ACTIVE, lesion deform the host bone but remain , lesion deform the host bone but remain contained in bone, require intralesional curettage.contained in bone, require intralesional curettage.

Stage 3-AGGRESSIVEStage 3-AGGRESSIVE , tumor extend beyond the bone, , tumor extend beyond the bone, require complete work-up and a removal with wide margins to require complete work-up and a removal with wide margins to avoid possible local recurrence. avoid possible local recurrence.

The staging system for The staging system for malignant tumorsmalignant tumors adopted by the adopted by the Musculoskeletal Tumor Society, and originally developed by Musculoskeletal Tumor Society, and originally developed by Enneking is based on the histological grade, the local extent, Enneking is based on the histological grade, the local extent, and the presence or absence of metastasis. and the presence or absence of metastasis.

Stage Grade Local Extent MetastasisStage Grade Local Extent Metastasis 1A low intracompartmental -1A low intracompartmental - 1B low extracompartmental -1B low extracompartmental - 2A high intracompartmental -2A high intracompartmental - 2B high extracompartmental -2B high extracompartmental - 3 any any +3 any any + Grade is determined by histological parameters. Low-grade Grade is determined by histological parameters. Low-grade

tumors generally have few mitotic figures, little if any cellular tumors generally have few mitotic figures, little if any cellular atypia, and have a relatively non-infiltrative growth pattern. atypia, and have a relatively non-infiltrative growth pattern. High-grade tumors tend to have marked cellular atypia, High-grade tumors tend to have marked cellular atypia, hyperchromatism, and nuclear pleomorphism. They often hyperchromatism, and nuclear pleomorphism. They often demonstrate an infiltrative growth patterndemonstrate an infiltrative growth pattern

OSTEOCHONDROMA OSTEOCHONDROMA Osteochondromas occur as solitary osteochondroma Osteochondromas occur as solitary osteochondroma (osteocartilaginous exostosis) or as multiple (osteocartilaginous exostosis) or as multiple osteochondromatosis.osteochondromatosis. Solitary OsteochondromaSolitary Osteochondroma is a developmental abnormality of is a developmental abnormality of bone. It occurs when part of the growth plate forms an bone. It occurs when part of the growth plate forms an outgrowth on the surface of the bone. This bone outgrowth may outgrowth on the surface of the bone. This bone outgrowth may or may not have a stalk. When a stalk is present, the structure or may not have a stalk. When a stalk is present, the structure is called pedunculated. When no stalk is present, it is called is called pedunculated. When no stalk is present, it is called sessile. sessile. An osteochondroma may grow in a child or adolescent. Its An osteochondroma may grow in a child or adolescent. Its growth usually stops at maturity. growth usually stops at maturity. Solitary osteochondromas are thought to be the most common Solitary osteochondromas are thought to be the most common benign bone tumor. benign bone tumor. It is seen in patients aged 10 to 30. It occurs equally in males 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 and females. It does not result from injury. It is thought to arise during skeletal growth when bone grows away from the growth during skeletal growth when bone grows away from the growth plate instead of in line with it. Cause of solitary osteochondroma plate instead of in line with it. Cause of solitary osteochondroma is unknown.is unknown.

SYMPTOMSSYMPTOMS

The most common symptom of an osteochondroma is The most common symptom of an osteochondroma is a painless mass near the joints. The knee and a painless mass near the joints. The knee and shoulder are more commonly involved. It can be found shoulder are more commonly involved. It can be found at the ends of any long bone and along the pelvis.at the ends of any long bone and along the pelvis. If the stalk of a pedunculated osteochondroma If the stalk of a pedunculated osteochondroma breaks, pain and swelling may start immediately. breaks, pain and swelling may start immediately. It can be located under a tendon. Snapping of the It can be located under a tendon. Snapping of the tissue over the tumor may cause activity-related pain. tissue over the tumor may cause activity-related pain. It can be located near a nerve or blood vessel, such It can be located near a nerve or blood vessel, such as behind the knee causing numbness and tingling in as behind the knee causing numbness and tingling in that extremity, periodic changes in blood flow, loss of that extremity, periodic changes in blood flow, loss of pulse or changes in color of the limb. pulse or changes in color of the limb.

DIAGNOSISDIAGNOSIS

A plain X-ray will show the bony growth.A plain X-ray will show the bony growth.

MRI scan may be used to look for MRI scan may be used to look for cartilage on the surface of the bony cartilage on the surface of the bony growth. Such cartilage in an adult patient growth. Such cartilage in an adult patient should be checked for cancer if it is >2 cm should be checked for cancer if it is >2 cm in size, or if there is pain. in size, or if there is pain.

CT scan may also be used. CT scan may also be used.

TREATMENTTREATMENTNonsurgical Treatment

Most of the time, solitary osteochondroma is not removed surgically. Observe it & take regular X-rays to keep track of any changes.

Surgical TreatmentWhen 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. Surgery may be considered if the osteochondroma: Is causing pain with activity Puts pressure on a nerve or blood vessel Has a large cap of cartilage The osteochondroma is removed at the level of the normal bone. Some of the inside of the bone may also be removed.

Osteochondromas are thought to be associated with a gene called EXT 1.

MULTIPLE MULTIPLE OSTEOCHONDROMATOSISOSTEOCHONDROMATOSIS

Multiple osteochondromatosis is also called multiple Multiple osteochondromatosis is also called multiple osteocartilaginous exostosis, multiple hereditary osteocartilaginous exostosis, multiple hereditary exostosis (MHE), familial osteochondromatosis, exostosis (MHE), familial osteochondromatosis, multiple hereditary osteochondromatosis, or multiple hereditary osteochondromatosis, or Diaphyseal aclasia. Diaphyseal aclasia. About 70 percent of the time, it is inherited. About 30 About 70 percent of the time, it is inherited. About 30 percent of the time, it occurs randomly. percent of the time, it occurs randomly. It affects males more often than females. It usually It affects males more often than females. It usually happens within the first three decades of life.happens within the first three decades of life.Alterations in genes called EXT genes are thought to Alterations in genes called EXT genes are thought to be the cause of this disease. It can be passed along in be the cause of this disease. It can be passed along in families. There is no other known risk factor or cause. families. There is no other known risk factor or cause. There is increased risk of malignant transformation. There is increased risk of malignant transformation.

SYMPTOMSSYMPTOMS

Symptoms are the same as solitary osteochondroma. Symptoms are the same as solitary osteochondroma. The number and location of osteochondromas varies. The number and location of osteochondromas varies. A patient may have deformities of the forearms, a A patient may have deformities of the forearms, a short stature, and knock-knees and ankles. This is short stature, and knock-knees and ankles. This is caused by abnormal growth in the bones affected by caused by abnormal growth in the bones affected by the osteochondromas. the osteochondromas.

Symptoms and signs of a tumor becoming cancerous Symptoms and signs of a tumor becoming cancerous are: are:

Growth of an osteochondroma after puberty Growth of an osteochondroma after puberty Pain at the site of an osteochondroma Pain at the site of an osteochondroma A cartilage cap >2cmA cartilage cap >2cm

DIAGNOSISDIAGNOSISIt is diagnosed when two or more osteochondromas It is diagnosed when two or more osteochondromas are seen on plain X-rays. Multiple are seen on plain X-rays. Multiple osteochondromatosis has been associated with EXT 1 osteochondromatosis has been associated with EXT 1 and EXT 2 genes. and EXT 2 genes. X-ray findings are the same as with solitary X-ray findings are the same as with solitary osteochondromas. osteochondromas. If cancer is suspected, you may need a thorough If cancer is suspected, you may need a thorough evaluation. This includes MRI & CT scans of the evaluation. This includes MRI & CT scans of the tumor. tumor. Pain at the site of a mass or growth after puberty Pain at the site of a mass or growth after puberty should be evaluated. The most common malignancy should be evaluated. The most common malignancy found in this transformation is called chondrosarcoma. found in this transformation is called chondrosarcoma. CT scan of the chest helps look for metastasis to the CT scan of the chest helps look for metastasis to the lungs.lungs. Biopsy can also be doneBiopsy can also be done

TREATMENTTREATMENT

Nonsurgical TreatmentNonsurgical TreatmentMost of the time, multiple osteochondromas are not Most of the time, multiple osteochondromas are not removed surgically. Tumors that do not show signs of removed surgically. Tumors that do not show signs of cancer are watched carefully. Deformities such as cancer are watched carefully. Deformities such as knock-knees or ankles may need surgery to straighten knock-knees or ankles may need surgery to straighten the bone. the bone.

Surgical TreatmentSurgical TreatmentThe tumor may be completely removed. This may also The tumor may be completely removed. This may also include taking out a whole portion of bone, which may include taking out a whole portion of bone, which may need to be replaced with a prosthesis.need to be replaced with a prosthesis. Chemotherapy and radiation therapy are not usually Chemotherapy and radiation therapy are not usually needed. needed.

UNICAMERAL BONE CYSTUNICAMERAL BONE CYST

A unicameral (simple) bone cyst is a cavity A unicameral (simple) bone cyst is a cavity found within a bone that is filled with straw-found within a bone that is filled with straw-colored fluid. It is a benign condition. colored fluid. It is a benign condition. UBC are usually found in patients younger than UBC are usually found in patients younger than 20 years of age.It occurs in one bone, in one 20 years of age.It occurs in one bone, in one location. The location of the cysts tends to be in location. The location of the cysts tends to be in proximal humerus or proximal femur. Less proximal humerus or proximal femur. Less common locations include the pelvis, ankle common locations include the pelvis, ankle (talus), or heel (calcaneus). (talus), or heel (calcaneus). There is no known cause. There are theories There is no known cause. There are theories involving a growth defect or disturbance at the involving a growth defect or disturbance at the epiphyseal plate leading to the formation of a epiphyseal plate leading to the formation of a unicameral bone cyst. unicameral bone cyst.

SYMPTOMSSYMPTOMS

Most unicameral bone cysts have no symptoms. Most unicameral bone cysts have no symptoms. Some are discovered incidentally. Other Some are discovered incidentally. Other unicameral bone cysts are not noticed at all. unicameral bone cysts are not noticed at all.

If a unicameral bone cyst is thinning the bone, If a unicameral bone cyst is thinning the bone, there may be pain with weightbearing activities.there may be pain with weightbearing activities.

If there is a pathologic fracture through the cyst, If there is a pathologic fracture through the cyst, the affected arm or leg may have pain, swelling, the affected arm or leg may have pain, swelling, and deformity. and deformity.

DIAGNOSISDIAGNOSIS

plain X-rays to show a hollow cavity in the plain X-rays to show a hollow cavity in the metaphyseal portion of the bone. metaphyseal portion of the bone. There is no associated bone expansion. A well-There is no associated bone expansion. A well-defined cortical rim can be seen. On rare defined cortical rim can be seen. On rare occasions, the cyst may cross the physis of an occasions, the cyst may cross the physis of an immature patient and extend into the epiphysis. immature patient and extend into the epiphysis. If the UBC is not typical in its appearance,CT & If the UBC is not typical in its appearance,CT & MRI may help determine the architecture of the MRI may help determine the architecture of the bone. bone.

TREATMENTTREATMENT

Nonsurgical TreatmentNonsurgical TreatmentA unicameral bone cyst without pain or other symptoms can be watched A unicameral bone cyst without pain or other symptoms can be watched with repeated X-rays with repeated X-rays

Surgical TreatmentSurgical TreatmentOnly unicameral bone cysts at risk of a pathologic fracture need treatment.Only unicameral bone cysts at risk of a pathologic fracture need treatment. If the unicameral bone cyst is painful, growing larger, or in a location that 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 may fracture, may be treated by aspiration and injecting it with a steroid or with demineralized bone from the bone bank, supplemented occasionally with demineralized bone from the bone bank, supplemented occasionally with bone marrow from the patient's pelvis. This method of treatment may with bone marrow from the patient's pelvis. This method of treatment may require more than one aspiration/injection. require more than one aspiration/injection. A unicameral bone cyst in a structurally compromising location may need A unicameral bone cyst in a structurally compromising location may need surgery including scraping the inside of the bone (curettage). The hole left 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 following this may require donor bone or a bone graft substitute to fill the defect. defect. Pathologic fractures occasionally incite a healing process. Depending on the Pathologic fractures occasionally incite a healing process. Depending on the size and location of the cyst, treatment with an internal fixation device may size and location of the cyst, treatment with an internal fixation device may also be needed. also be needed.

OSTEOID OSTEOMAOSTEOID OSTEOMA      Osteoid Osteoma is a benign bone lesion with a nidus of <2 Osteoid Osteoma is a benign bone lesion with a nidus of <2 cm surrounded by a zone of reactive bone. The tumor occurs cm surrounded by a zone of reactive bone. The tumor occurs most frequently in the second decade and affects males twice most frequently in the second decade and affects males twice as often as females. as often as females. The proximal femur is the most common location followed by The proximal femur is the most common location followed by the tibia, posterior elements of the spine, and the humerus.It is the tibia, posterior elements of the spine, and the humerus.It is found in the diaphysis or the metaphysis of the proximal end of found in the diaphysis or the metaphysis of the proximal end of the bone.the bone.

SYMPTOMS SYMPTOMS :-:-It has a distinct clinical picture of dull pain that is worse at night 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-and disappears within 20 to 30 minutes of treatment with non-steroidal anti-inflammatory medication.steroidal anti-inflammatory medication. Joint pain may be present with a periarticular lesion and Joint pain may be present with a periarticular lesion and synovitis can occur secondary to an intraarticular lesion. Local synovitis can occur secondary to an intraarticular lesion. Local symptoms can include an increase in skin temperature, symptoms can include an increase in skin temperature, increased sweating and tenderness. Epiphyseal lesions can increased sweating and tenderness. Epiphyseal lesions can cause abnormal growth.cause abnormal growth.

DIAGNOSISDIAGNOSIS

The classic radiological presentation of an osteoid osteoma is a The classic radiological presentation of an osteoid osteoma is a radiolucent nidus surrounded by a dramatic reactive sclerosis in radiolucent nidus surrounded by a dramatic reactive sclerosis in the cortex of the bone. The center can range from partially the cortex of the bone. The center can range from partially mineralized to osteolytic to entirely calcified. The lesion can mineralized to osteolytic to entirely calcified. The lesion can occur only in the cortex, in both the cortex and medulla, or only occur only in the cortex, in both the cortex and medulla, or only the medulla. The reactive sclerosis may be present or absent. the medulla. The reactive sclerosis may be present or absent. The four diagnostic features includeThe four diagnostic features include

(1) a sharp round or oval lesion that is(1) a sharp round or oval lesion that is (2) less than 2 cm in diameter, (2) less than 2 cm in diameter, (3) has a homogeneous dense center and(3) has a homogeneous dense center and (4) a 1-2 mm peripheral radiolucent zone.' (4) a 1-2 mm peripheral radiolucent zone.'    CT is the preferred method of evaluation, especially if the CT is the preferred method of evaluation, especially if the

lesion is in the spine or obscured by reactive sclerosis. The lesion is in the spine or obscured by reactive sclerosis. The radiologic D/D includes osteoblastoma, osteomyelitis, arthritis, radiologic D/D includes osteoblastoma, osteomyelitis, arthritis, stress fracture and enostosis stress fracture and enostosis

TREATMENTTREATMENT

Osteoid osteoma will resolve without Osteoid osteoma will resolve without treatment in an average of 33 months.treatment in an average of 33 months. If the patient does not wish to endure the If the patient does not wish to endure the pain and prolonged use of non-steroidal pain and prolonged use of non-steroidal anti-inflammatory medications, surgical anti-inflammatory medications, surgical removal or percutaneous ablation of the removal or percutaneous ablation of the nucleus is indicated.nucleus is indicated.

CHONDROBLASTOMACHONDROBLASTOMA

Sometimes called Sometimes called Codman's tumorCodman's tumor, a chondroblastoma is , a chondroblastoma is a rare type of benign bone tumor that originates from a rare type of benign bone tumor that originates from cartilage. Chondroblastoma most often affects the ends of cartilage. Chondroblastoma most often affects the ends of the long bones in the arms and legs at the hip, shoulder, the long bones in the arms and legs at the hip, shoulder, and knee.and knee.Chondroblastoma is a rare bone tumor that can affect Chondroblastoma is a rare bone tumor that can affect people of all ages, most common between the ages of 10 people of all ages, most common between the ages of 10 and 20 years. This type of tumor is also more common in and 20 years. This type of tumor is also more common in males than females.males than females.

The exact cause of chondroblastoma is not known. The The exact cause of chondroblastoma is not known. The tumors are believed to originate from immature cartilage tumors are believed to originate from immature cartilage producing cells called chondroblasts.producing cells called chondroblasts.

SYMPTOMSSYMPTOMS

Symptoms of chondroblastoma may vary Symptoms of chondroblastoma may vary depending on the location of the tumor. However, depending on the location of the tumor. However, each individual may experience symptoms each individual may experience symptoms differently. Symptoms may include:differently. Symptoms may include:

pain in the knee, hip, and shoulder joint. pain in the knee, hip, and shoulder joint. withered or shrunken appearance of the muscle near withered or shrunken appearance of the muscle near

the affected bone the affected bone impaired mobility of the adjacent joint impaired mobility of the adjacent joint fluid accumulation in the joint adjacent to the affected fluid accumulation in the joint adjacent to the affected

bone bone

DIAGNOSISDIAGNOSISX-RAY : The diagnosis of chondroblastoma can X-RAY : The diagnosis of chondroblastoma can usually be made by radiograph.The most common site usually be made by radiograph.The most common site for chondroblastoma is the epiphysis. The lesion is for chondroblastoma is the epiphysis. The lesion is lytic with well defined margins and can be from 1-6cm lytic with well defined margins and can be from 1-6cm in size. Scalloping or expansion of cortical bone may in size. Scalloping or expansion of cortical bone may be present. Fine calcifications, either punctate or in be present. Fine calcifications, either punctate or in rings, may be visible.rings, may be visible.MRI & CT : Cysts are present about 20% of the time MRI & CT : Cysts are present about 20% of the time and both MRI and CT can define the fluid levels. CT is and both MRI and CT can define the fluid levels. CT is also useful for defining the relationship of the tumor to also useful for defining the relationship of the tumor to the joint, integrity of the cortex, and intralesional the joint, integrity of the cortex, and intralesional calcifications. calcifications. The differential diagnosis includes enchondroma, The differential diagnosis includes enchondroma, central chondrosarcoma and aneurysmal bone cyst.central chondrosarcoma and aneurysmal bone cyst.

Chondroblastoma is made up of uniform, Chondroblastoma is made up of uniform, polygonal cells that are closely packed. These polygonal cells that are closely packed. These primitive cells are derived from the epiphyseal primitive cells are derived from the epiphyseal cartilage plate and have abundant cytoplasm. cartilage plate and have abundant cytoplasm. There is little mitotic activity. A scant chondroid There is little mitotic activity. A scant chondroid matrix may be superimposed by a pericellular matrix may be superimposed by a pericellular deposit of calcification that appears like "deposit of calcification that appears like "chicken-wirechicken-wire". Giant cells are often present.". Giant cells are often present.

TREATMENTTREATMENT

The goal for treatment of chondroblastoma is to remove The goal for treatment of chondroblastoma is to remove the tumor and prevent damage to the end of the affected the tumor and prevent damage to the end of the affected bone. bone. Treatment may include:Treatment may include:

Surgical removal of the tumor -Surgical removal of the tumor -Biopsy and curettage with possible use of  adjuvant liquid Biopsy and curettage with possible use of  adjuvant liquid nitrogen or phenol, or a mechanical burr. It may be necessary nitrogen or phenol, or a mechanical burr. It may be necessary to reconstruct articular surfaces due to subchondral erosion.to reconstruct articular surfaces due to subchondral erosion.

Any joint invasion is usually secondary to previous Any joint invasion is usually secondary to previous instrumentation. All pulmonary nodules should be excised.instrumentation. All pulmonary nodules should be excised.

Bone grafting - a surgical procedure in which healthy bone is Bone grafting - a surgical procedure in which healthy bone is transplanted from another part of the patient's body into the transplanted from another part of the patient's body into the affected area, if necessary, to repair damaged bone. affected area, if necessary, to repair damaged bone.

Physical therapy (to restore strength and function after surgery) Physical therapy (to restore strength and function after surgery)

CHORDOMACHORDOMA Chordoma is a rare tumor that usually occurs in the spine and Chordoma is a rare tumor that usually occurs in the spine and base of the skull. base of the skull. It is a malignant tumor that grows fairly slowly. It can spread to It is a malignant tumor that grows fairly slowly. It can spread to other organs, usually the lungs. It represents only about 1 other organs, usually the lungs. It represents only about 1 percent of all malignant bone tumors. percent of all malignant bone tumors. Chordomas develop in a structure called the notochord. The Chordomas develop in a structure called the notochord. The notochord forms the early spine in the beginning stages of fetal notochord forms the early spine in the beginning stages of fetal development. Most of the notochord is replaced by the spine development. Most of the notochord is replaced by the spine during the first six months of development. Small areas can during the first six months of development. Small areas can remain, and chordomas can form in these areas. remain, and chordomas can form in these areas. Most chordomas occur at the base of the spine (sacrum), in the Most chordomas occur at the base of the spine (sacrum), in the tailbone (coccyx) or at the base of the skull (40 percent).tailbone (coccyx) or at the base of the skull (40 percent).between 40 and 70 years of age, Occasionally in younger between 40 and 70 years of age, Occasionally in younger patients, even children. patients, even children. Chordomas are life-threatening. They can cause death by direct Chordomas are life-threatening. They can cause death by direct growth or by spreading to other organs. They spread to the growth or by spreading to other organs. They spread to the lungs about 20 to 30 percent of the time. lungs about 20 to 30 percent of the time.

RISK FACTORSRISK FACTORS

Chordomas occur spontaneously. Chordomas occur spontaneously.

They are not known to be caused by They are not known to be caused by trauma, environmental factors, or diet.trauma, environmental factors, or diet.

Chordomas are not inherited,not Chordomas are not inherited,not associated with any medical conditions or associated with any medical conditions or use of any medications or supplements. use of any medications or supplements.

SYMPTOMSSYMPTOMSSymptoms depend on the location of the tumor. Symptoms depend on the location of the tumor. Tumors in the skull can cause headache or Tumors in the skull can cause headache or problems with vision. problems with vision. Tumors in the spine can cause pain in the area Tumors in the spine can cause pain in the area of tumor (neck, back, or tailbone). A tumor of tumor (neck, back, or tailbone). A tumor pressing on nearby nerves can cause pressing on nearby nerves can cause symptoms similar to a "slipped" (herniated) symptoms similar to a "slipped" (herniated) disk. These symptoms include arm or leg pain, disk. These symptoms include arm or leg pain, weakness, or numbness. weakness, or numbness. Tumors in the tailbone area can cause a Tumors in the tailbone area can cause a noticeable mass, numbness in the crotch area, noticeable mass, numbness in the crotch area, and problems with the bowel and bladder. and problems with the bowel and bladder.

DIAGNOSISDIAGNOSISX-rays of the sacrum are often difficult to interpret. X-rays of the sacrum are often difficult to interpret.

The plain radiographic hallmark of a chordoma is midline bony destruction The plain radiographic hallmark of a chordoma is midline bony destruction with a large associated soft-tissue mass with a large associated soft-tissue mass

In the vertebral body, the chordoma is lytic, centrally located, and slowly In the vertebral body, the chordoma is lytic, centrally located, and slowly expansile. Areas of sclerosis due to reactive bone formation are seen. expansile. Areas of sclerosis due to reactive bone formation are seen. Adjacent vertebral bodies and the intervening disc space can be involved.Adjacent vertebral bodies and the intervening disc space can be involved.Reports vary as to the activity of a chordoma on bone scan, and Reports vary as to the activity of a chordoma on bone scan, and accumulation of isotope in the bladder can obscure the sacral area. accumulation of isotope in the bladder can obscure the sacral area. CT scans and MRI - extremely helpful in determining the extent of the lesion CT scans and MRI - extremely helpful in determining the extent of the lesion and its proximity to vital structures. This is essential in preoperative and its proximity to vital structures. This is essential in preoperative planning. CT scans identify calcified areas that are not evident on plain planning. CT scans identify calcified areas that are not evident on plain films. Along with myelography, CT is helpful in planning resection of a films. Along with myelography, CT is helpful in planning resection of a vertebral lesion. vertebral lesion. MRI is useful in discovering recurrent nodules after surgical resection.MRI is useful in discovering recurrent nodules after surgical resection.Angiography is only occasionally indicated to identify the proximity of a Angiography is only occasionally indicated to identify the proximity of a cervical chordoma to the vertebral arteries. cervical chordoma to the vertebral arteries. The radiographic differential diagnosis includes metastatic disease, multiple The radiographic differential diagnosis includes metastatic disease, multiple myeloma, giant cell tumor, and neurogenic tumors. myeloma, giant cell tumor, and neurogenic tumors.

TREATMENTTREATMENT Treatment of chordomas is very difficult. This is mainly because Treatment of chordomas is very difficult. This is mainly because

these tumors are near the brain and spinal cord. these tumors are near the brain and spinal cord. Traditional chemotherapy has not been very effective. Traditional chemotherapy has not been very effective.

Surgery is preferred, where possible. Radiation in combination Surgery is preferred, where possible. Radiation in combination with surgery is often used to treat these tumors. Radiation with surgery is often used to treat these tumors. Radiation alone rarely cures the tumor. When tumors cannot be surgically alone rarely cures the tumor. When tumors cannot be surgically removed, sometimes radiation therapy can stop the growth of removed, sometimes radiation therapy can stop the growth of the tumor. the tumor. The surgeon must remove the tumor and a margin of normal The surgeon must remove the tumor and a margin of normal tissue around it. This often results in a loss of neurologic tissue around it. This often results in a loss of neurologic function. For example, surgical removal of tumors in the sacrum function. For example, surgical removal of tumors in the sacrum can result in loss of control of bowel and bladder function can result in loss of control of bowel and bladder function (incontinence). (incontinence). New drug treatments, including use of a drug called imatinib New drug treatments, including use of a drug called imatinib mseylate are being investigated which may help slow the mseylate are being investigated which may help slow the growth of tumors that can't be removed. growth of tumors that can't be removed.

ENCHONDROMA ENCHONDROMA Enchondroma is one type of benign cartilage tumor that appears on Enchondroma is one type of benign cartilage tumor that appears on the inside of the bone. These tumors usually begin and grow in the inside of the bone. These tumors usually begin and grow in childhood, then stop growing but remain present throughout childhood, then stop growing but remain present throughout adulthood. They are often found in patients between 10 and 20 years of adulthood. They are often found in patients between 10 and 20 years of age. Some cases become dormant or burned out. age. Some cases become dormant or burned out. very common in the small bones of the hand and feet. In fact, they are 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 the most common tumor of the hand. They also occur in the long bones of the upper arm and thigh.bones of the upper arm and thigh.In rare cases, multiple tumors can appear as part of a syndrome. These In rare cases, multiple tumors can appear as part of a syndrome. These syndromes are Ollier's disease and Maffucci's syndrome. Single syndromes are Ollier's disease and Maffucci's syndrome. Single enchondromas can become cancerous, but this is very rare. The rate 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 of change to a cancerous tumor is a little higher in Ollier's disease and Maffucci's syndrome. Maffucci's syndrome. The cause of enchondromas is not completely clear, but they don't The cause of enchondromas is not completely clear, but they don't seem to be caused by exposure to chemicals or radiation or patient seem to be caused by exposure to chemicals or radiation or patient activities. activities. The chance that a single one of these tumors will become malignant is 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 very low. For the very few that become cancer, when they become malignant, they usually become a chondrosarcoma. malignant, they usually become a chondrosarcoma.

SYMPTOMSSYMPTOMSEnchondromas are usually painless,found during an Enchondromas are usually painless,found during an X-ray evaluation of another problem in the same area. X-ray evaluation of another problem in the same area. When these tumors appear in the hands or feet, or in When these tumors appear in the hands or feet, or in multiple lesions, they can deform the bone, lead to multiple lesions, they can deform the bone, lead to pathologic fracture. pathologic fracture.

The deformities in patients with Ollier's disease and The deformities in patients with Ollier's disease and Maffucci's syndrome can be quite severe. Maffucci's syndrome can be quite severe.

If pain from other nearby sources has been excluded, If pain from other nearby sources has been excluded, the tumor should be carefully studied to determine the tumor should be carefully studied to determine whether it could be a low-grade chondrosarcoma. Pain whether it could be a low-grade chondrosarcoma. Pain at night or at rest is more likely a malignant tumor but at night or at rest is more likely a malignant tumor but pain is also very common with some shoulder pain is also very common with some shoulder conditions like rotator cuff tendonitis. conditions like rotator cuff tendonitis.

Diagnosis Diagnosis Most enchondromas are found when X-rays are taken for another reason, 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 such as injury or arthritis. The diagnosis of an enchondroma is important to make sure it is not a more aggressive or cancerous tumor. make sure it is not a more aggressive or cancerous tumor. It is important to know if the tumor is painful and the kind of pain it causes. 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 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. 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 Often pain from a different reason in a nearby joint may have prompted the X-ray. X-ray. on the X-rays. Enchondromas appear as small (less than 5 cm) lobe-shaped 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 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. show a pattern of rings and arcs that indicates the tumor contains cartilage. Characteristics of a more aggressive tumor or malignant chondrosarcoma Characteristics of a more aggressive tumor or malignant chondrosarcoma include: include:

Scalloping of the inner surface of the bone Scalloping of the inner surface of the bone Thickening of the bone cortex Thickening of the bone cortex Destruction of the bone by the tumor Destruction of the bone by the tumor Soft-tissue mass Soft-tissue mass Bone erosion that is growing Bone erosion that is growing Erosion surrounded by reactive bone Erosion surrounded by reactive bone

Most enchondromas are diagnosed on plain X-rays.Most enchondromas are diagnosed on plain X-rays.

CT or MRI scan give a more complete picture of the CT or MRI scan give a more complete picture of the bone around the tumor. .bone around the tumor. .

Bone scans - both benign and malignant tumors can Bone scans - both benign and malignant tumors can show increased uptake of the radioactive tracer used show increased uptake of the radioactive tracer used to detect increased bone activity. to detect increased bone activity.

Under the microscope, enchondromas have islands of Under the microscope, enchondromas have islands of cartilage that surrounds them. Usually, cartilage is not cartilage that surrounds them. Usually, cartilage is not found in the center of bones. However, these tumors found in the center of bones. However, these tumors in the hand and foot or in Ollier's disease or Maffucci's in the hand and foot or in Ollier's disease or Maffucci's syndrome may contain more odd-looking cartilage. syndrome may contain more odd-looking cartilage.

Low-grade chondrosarcomas (cancers) look more Low-grade chondrosarcomas (cancers) look more cellular under a microscope than enchondromas and cellular under a microscope than enchondromas and there is less normal bone in the tumor. there is less normal bone in the tumor.

TREATMENTTREATMENT Nonsurgical TreatmentNonsurgical Treatment

Most enchondromas require no treatment at all. Most enchondromas require no treatment at all. When needed, treatment for enchondroma can When needed, treatment for enchondroma can vary due to the difficulty in determining the vary due to the difficulty in determining the aggressiveness of the lesion. aggressiveness of the lesion.

Stage 1 latent lesions - that show no symptoms Stage 1 latent lesions - that show no symptoms can be watched with regular X-rays to ensure can be watched with regular X-rays to ensure the tumor is not growing. Most surgeons think the tumor is not growing. Most surgeons think that tumors without symptoms do not need to that tumors without symptoms do not need to be removed. be removed.

Surgical TreatmentSurgical Treatment

Sx Tt is usually with scraping out and filling of the cavity Sx Tt is usually with scraping out and filling of the cavity with bone graft or other filling substances. Although they with bone graft or other filling substances. Although they can come back (recur), most of them will not. can come back (recur), most of them will not. Tumors that cause pathologic fractures are usually treated Tumors that cause pathologic fractures are usually treated by allowing the fracture to heal. Then, the tumor is scraped by allowing the fracture to heal. Then, the tumor is scraped out to prevent another fracture. out to prevent another fracture. More aggressive tumors with bone destruction or with a More aggressive tumors with bone destruction or with a mass growing outside the bone are usually mass growing outside the bone are usually chondrosarcomas. These tumors need to be removed. chondrosarcomas. These tumors need to be removed. Malignant tumors are either scraped out or the entire bone Malignant tumors are either scraped out or the entire bone around the lesion must be removed. This decision is made around the lesion must be removed. This decision is made depending on the grade of the tumor. The grade of the depending on the grade of the tumor. The grade of the tumor is determined by imaging studies and biopsy. tumor is determined by imaging studies and biopsy. Unfortunately, a biopsy will not often help in these cases. Unfortunately, a biopsy will not often help in these cases. Even specialized bone pathologists can have trouble Even specialized bone pathologists can have trouble determining the difference between a benign enchondroma determining the difference between a benign enchondroma and a low grade chondrosarcoma. and a low grade chondrosarcoma.

GIANT CELL TUMORGIANT CELL TUMORGiant cell tumor of bone is a rare, aggressive non-cancerous (benign) Giant cell tumor of bone is a rare, aggressive non-cancerous (benign) tumor.tumor.occurs in adults between the ages of 20 and 40 years. very rarely seen in 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 children or in adults older than 65 years of age. Giant cell tumors occur in approximately one person per million per year.approximately one person per million per year.Giant cell tumors are named for the way they look under the microscope. Giant cell tumors are named for the way they look under the microscope. Many "giant cells" are seen. They are formed by fusion of several individual Many "giant cells" are seen. They are formed by fusion of several individual cells into a single, larger complex. cells into a single, larger complex. Many bone tumors and other conditions (including normal bone) contain Many bone tumors and other conditions (including normal bone) contain giant cells. Giant cell tumor of bone is given its characteristic appearance by 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 the constant finding of a large number of these cells existing in a typical background.background.Giant cell tumor of bone occurs almost exclusively in the end portion of long 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 bones next to the joints (epiphysis). In rare cases, this tumor may spread to the lungs. the lungs. Giant cell tumors of bone occur spontaneously. They are not known to be Giant cell tumors of bone occur spontaneously. They are not known to be associated with trauma, environmental factors, or diet. They are not associated with trauma, environmental factors, or diet. They are not inherited. inherited. In rare cases, they may be associated with hyperparathyroidism. In rare cases, they may be associated with hyperparathyroidism.

Giant Cell Tumor Osteoclastoma of Bone in RibGiant Cell Tumor Osteoclastoma of Bone in Rib"Soap Bubble" appearance on X-ray"Soap Bubble" appearance on X-ray

SYMPTOMSSYMPTOMS

GCT most frequently occur around the knee joint in GCT most frequently occur around the knee joint in the lower end of the femur or the upper end of the the lower end of the femur or the upper end of the tibia. Other common locations include the wrist (lower tibia. Other common locations include the wrist (lower end of the radius), the hip (upper end of the femur), end of the radius), the hip (upper end of the femur), the shoulder (upper end of the humerus), and lower the shoulder (upper end of the humerus), and lower back (connection of the spine and pelvis).back (connection of the spine and pelvis).The bone involved is generally tender. There may be The bone involved is generally tender. There may be swelling in the area around it. swelling in the area around it. The first symptom is pain in the area of the tumor. The first symptom is pain in the area of the tumor. There may be pain with movement of a nearby joint. There may be pain with movement of a nearby joint. Pain generally increases with activity and decreases Pain generally increases with activity and decreases with rest. Pain is usually mild initially, but it with rest. Pain is usually mild initially, but it progressively increases. Occasionally the bone progressively increases. Occasionally the bone weakened by the tumor may break and cause sudden weakened by the tumor may break and cause sudden onset of severe pain. onset of severe pain.

DIAGNOSISDIAGNOSISGiant cell tumors appear on X-rays as Giant cell tumors appear on X-rays as destructive (lytic) lesions next to a joint. destructive (lytic) lesions next to a joint. Occasionally, the involved area of bone can be Occasionally, the involved area of bone can be surrounded by a thin rim of white bone that may surrounded by a thin rim of white bone that may be complete or incomplete. The bone in the be complete or incomplete. The bone in the area of the tumor is sometimes expanded. area of the tumor is sometimes expanded. MRI and CT scans better evaluate the area of MRI and CT scans better evaluate the area of involvement.involvement.bone scan generally shows a "hot spot" in the bone scan generally shows a "hot spot" in the bone where the tumor is. An X-ray or CT scan bone where the tumor is. An X-ray or CT scan of the chest will often be done to look for of the chest will often be done to look for possible spread to the lungspossible spread to the lungs

TreatmentTreatmentIf not treated, these tumors will continue to grow and destroy bone. Surgery 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. has proven to be the most effective treatment for giant cell tumors.

Nonsurgical TreatmentNonsurgical TreatmentRadiation therapy is a nonsurgical effective treatment option. However, this 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 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 who receive it. Therefore, radiation therapy is used only in the most difficult cases where surgery cannot be performed safely or effectively. cases where surgery cannot be performed safely or effectively. Some centers have reported successful treatment of giant cell tumors with Some centers have reported successful treatment of giant cell tumors with Embolization- a process where the blood vessels supplying the tumor are Embolization- a process where the blood vessels supplying the tumor are occluded by means of a catheter threaded through the main arteries. 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 Blocking the blood supply to the tumor can cause it to shrink and even disappear because the tumor loses its source of nutrition. disappear because the tumor loses its source of nutrition. Clinical trials of Drug interferon are currently underway. works by interfering Clinical trials of Drug interferon are currently underway. works by interfering with the ability of the tumor to grow new blood vessels. Because tumors 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 require an ever-increasing blood supply to grow, interferon may also cause the tumor to shrink or even disappear. the tumor to shrink or even disappear. Like radiation therapy,, embolization and treatment with interferon are Like radiation therapy,, embolization and treatment with interferon are generally reserved for those tumors that are difficult to remove surgically or generally reserved for those tumors that are difficult to remove surgically or in situations where the tumor keeps returning despite treatment or if it in situations where the tumor keeps returning despite treatment or if it spreads. spreads.

Surgical TreatmentSurgical TreatmentIf treatment is limited to curettage and bone grafting alone, the tumor may 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 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.rather than bone graft results in a lower rate of recurrence.Scooping out the tumor (curettage) is generally performed. This techniques 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 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).of the patient's own body (autograft) or from a cadaver (allograft).Enhancing the curettage with a high-speed burr or with the use of agents Enhancing the curettage with a high-speed burr or with the use of agents such as liquid nitrogen, hydrogen peroxide, or phenol, followed by such as liquid nitrogen, hydrogen peroxide, or phenol, followed by placement of bone cement decreases the recurrence rate to 10 percent to placement of bone cement decreases the recurrence rate to 10 percent to 29 percent.29 percent. More complex tumor removal and reconstruction is sometimes necessary in More complex tumor removal and reconstruction is sometimes necessary in situations where the tumor has caused excessive damage or recurred.situations where the tumor has caused excessive damage or recurred. In the rare cases where the tumor has spread to the lungs, surgical removal 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. of the tumor in the lungs results in cure 75 percent of the time.

MULTIPLE MYELOMA/ MULTIPLE MYELOMA/ PLASMACYTOMAPLASMACYTOMA

CausesCauses

MM may occur spontaneouslyMM may occur spontaneously

On exposure to ionizing radiation and the On exposure to ionizing radiation and the pesticide dioxinpesticide dioxin

Infection with some viruses (HIV and Infection with some viruses (HIV and human herpes 8) has also been human herpes 8) has also been associated with multiple myeloma. associated with multiple myeloma.

No known risk factors are inherited. No known risk factors are inherited.

SYMPTOMSSYMPTOMSusually bone pain is main complain. Other symptoms include: usually bone pain is main complain. Other symptoms include:

Fatigue Fatigue Feeling ill Feeling ill Fever Fever Night sweats Night sweats

Weight loss is not common in the early stages.Weight loss is not common in the early stages.patients are pale with diffuse bone tenderness, especially around the patients are pale with diffuse bone tenderness, especially around the sternum (breastbone) and pelvis (hips). sternum (breastbone) and pelvis (hips). spine is the most common location for a pathological fracture. It can also spine is the most common location for a pathological fracture. It can also happen in the ribs and pelvis. happen in the ribs and pelvis. Compression of the spinal cord in 10%-15%This causes pain in the back Compression of the spinal cord in 10%-15%This causes pain in the back and legs and numbness and weakness in the legs. and legs and numbness and weakness in the legs. Patients who have high levels of calcium in the blood may experience Patients who have high levels of calcium in the blood may experience nausea, fatigue, confusion, constipation, and frequent urination. nausea, fatigue, confusion, constipation, and frequent urination. Patients with anemia may experience fatigue, weakness, and shortness of Patients with anemia may experience fatigue, weakness, and shortness of breath with exercise. breath with exercise. In advanced cases, patients typically have recurrent infections and can have In advanced cases, patients typically have recurrent infections and can have kidney failure. kidney failure.

DIAGNOSISDIAGNOSISIn addition to a complete medical history and physical examination, In addition to a complete medical history and physical examination, diagnostic procedures for myeloma bone disease may include-diagnostic procedures for myeloma bone disease may include-x-rayx-ray - a diagnostic test - a diagnostic test

When only one lesion is found When only one lesion is found it is called a plasmacytoma, it is called a plasmacytoma, Most doctors believe plasmacytomaMost doctors believe plasmacytoma is simply an early, isolated form of is simply an early, isolated form of multiple myeloma.multiple myeloma.

blood and urine testsblood and urine tests bone marrow aspiration and/or biopsybone marrow aspiration and/or biopsy - a procedure that involves taking - a procedure that involves taking a small amount of bone marrow fluid (aspiration) and/or solid bone marrow 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 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. the number, size, and maturity of blood cells and/or abnormal cells. magnetic resonance imaging (MRI)magnetic resonance imaging (MRI) - a diagnostic procedure. - a diagnostic procedure.

CHEMOTHERAPYCHEMOTHERAPY

The standard treatment medications are The standard treatment medications are melphalan and prednisone. melphalan and prednisone.

The median survival rate is three years with this The median survival rate is three years with this treatment alone. For patients in whom this treatment alone. For patients in whom this therapy is ineffective, alternatives include: therapy is ineffective, alternatives include:

VBMCP (vincristine, carmustine, melphalan, VBMCP (vincristine, carmustine, melphalan, cyclophosphamide and prednisone) cyclophosphamide and prednisone)

VAD (vincristine, adriamycin and VAD (vincristine, adriamycin and dexamethasone) dexamethasone)

a recent advancement in the treatment of multiple a recent advancement in the treatment of multiple myeloma has increased, response rates and survival.myeloma has increased, response rates and survival.

This treatment consists of high-dose chemotherapy, This treatment consists of high-dose chemotherapy, followed by autologous stem cell transplantation. With followed by autologous stem cell transplantation. With this treatment, patients have a 20 percent chance of this treatment, patients have a 20 percent chance of living longer than 10 years. This stem cell living longer than 10 years. This stem cell transplantation involves: transplantation involves:

Harvesting a patient's own blood cells Harvesting a patient's own blood cells

Conditioning them with very high doses of melphalan Conditioning them with very high doses of melphalan

Re-infusing the blood cells back into the patient Re-infusing the blood cells back into the patient

RADIATION THERAPYRADIATION THERAPY

Radiation therapy is reserved for Radiation therapy is reserved for decreasing the size of symptomatic bone decreasing the size of symptomatic bone lesions. lesions.

SUPPORTIVE CARESUPPORTIVE CARESupportive care is critical. Supportive care includes managing Supportive care is critical. Supportive care includes managing the bone disease, anemia, infections, kidney failure, and pain the bone disease, anemia, infections, kidney failure, and pain associated with multiple myeloma. associated with multiple myeloma. Bisphosphonates (medication) can prevent destructive bone Bisphosphonates (medication) can prevent destructive bone lesions and spine fractures. lesions and spine fractures. Erythropoetin or occasional blood transfusions can manage Erythropoetin or occasional blood transfusions can manage anemia. anemia. Antibody infusions and vaccinations can help patients with Antibody infusions and vaccinations can help patients with recurrent infections. recurrent infections. Corticosteroids and hydration can be used to treat high blood Corticosteroids and hydration can be used to treat high blood calcium concentrations (from bone loss) and dehydration. calcium concentrations (from bone loss) and dehydration. Narcotics can decrease the pain associated with bone lesions. Narcotics can decrease the pain associated with bone lesions. Operative intervention may be required to stabilize and control Operative intervention may be required to stabilize and control the pain associated with bone fractures. the pain associated with bone fractures.

SURGICAL TREATMENTSURGICAL TREATMENT

Surgery will not cure multiple myeloma. Surgery Surgery will not cure multiple myeloma. Surgery is used to treat fractures and impending is used to treat fractures and impending fractures in the spine, pelvis, hip, and shoulder. fractures in the spine, pelvis, hip, and shoulder. The goal of these surgeries is to decrease pain The goal of these surgeries is to decrease pain and maintain function. and maintain function. Internal fixation augmented with cement is Internal fixation augmented with cement is frequently recommended, as are joint frequently recommended, as are joint replacements and vertebroplasties (for spine replacements and vertebroplasties (for spine fractures). fractures). Operative intervention does not alter the survival Operative intervention does not alter the survival rate, but it does increase the quality of life. rate, but it does increase the quality of life.

EWING’S SARCOMAEWING’S SARCOMAEwing's sarcoma is a malignant tumor. usually begins growing Ewing's sarcoma is a malignant tumor. usually begins growing initially in a bone. Occasionally, it also begins in the soft tissues initially in a bone. Occasionally, it also begins in the soft tissues (extraosseous Ewing's sarcoma). (extraosseous Ewing's sarcoma). Unlike most cancers that are known to arise from a certain kind Unlike most cancers that are known to arise from a certain kind of tissue or organ for example, osteosarcoma arises from bone of tissue or organ for example, osteosarcoma arises from bone cells, and breast carcinoma arises from breast cells. The exact cells, and breast carcinoma arises from breast cells. The exact cell of origin for Ewing's sarcoma is not known. cell of origin for Ewing's sarcoma is not known. Recently,the disease has been defined to include a family of Recently,the disease has been defined to include a family of tumors. This includes the Ewing's sarcoma of bone or soft tumors. This includes the Ewing's sarcoma of bone or soft tissue, and primitive neuroectodermal tumor (PNET). tissue, and primitive neuroectodermal tumor (PNET). Sometimes these tumors are called small blue cell (round cell) Sometimes these tumors are called small blue cell (round cell) tumors. They are named for the way they look under a tumors. They are named for the way they look under a microscope. microscope. all malignant bone tumors are rare. Among malignant bone all malignant bone tumors are rare. Among malignant bone tumors in children and young adults, Ewing's sarcoma is the tumors in children and young adults, Ewing's sarcoma is the second most common. There is no known cause. second most common. There is no known cause. There are no known risk factors for developing Ewing's There are no known risk factors for developing Ewing's sarcoma. There is no known genetic link. sarcoma. There is no known genetic link.

SYMPTOMS SYMPTOMS

There is usually pain and/or swelling at the There is usually pain and/or swelling at the affected site. A bone weakened by disease may affected site. A bone weakened by disease may break after a minor injury. break after a minor injury.

Ewing's sarcoma usually affects the long Ewing's sarcoma usually affects the long bones, such as the femur, tibia, and humerus. bones, such as the femur, tibia, and humerus. The bones of the pelvis may also be affected. The bones of the pelvis may also be affected.

DIAGNOSISDIAGNOSIS

These include X-rays, MRI , CT and bone scans. These include X-rays, MRI , CT and bone scans. The diagnosis is confirmed by biopsy. This may be The diagnosis is confirmed by biopsy. This may be done in an operating room or X-ray department. done in an operating room or X-ray department. Special tests can help to diagnose Ewing's sarcoma. Special tests can help to diagnose Ewing's sarcoma. The hallmark is an abnormality in the cell's The hallmark is an abnormality in the cell's chromosomes. Genetic material is mismatched. chromosomes. Genetic material is mismatched. Chromosomes #11 and #22 are affected. Chromosomes #11 and #22 are affected. Blood tests, lung CT scan, bone scan, and bone Blood tests, lung CT scan, bone scan, and bone marrow biopsy are needed to show the extent of marrow biopsy are needed to show the extent of disease. This process is known as "staging." disease. This process is known as "staging." Tests can show if cancer has spread to other sites. Tests can show if cancer has spread to other sites. such as the lungs or bone marrow. such as the lungs or bone marrow.

TREATMENTTREATMENT

Ewing's sarcoma is usually diagnosed as a primary Ewing's sarcoma is usually diagnosed as a primary bone tumor without evidence of spread elsewhere. bone tumor without evidence of spread elsewhere. Even if tests to do not show spread, the treatment Even if tests to do not show spread, the treatment strategy assumes that a very small amount of spread strategy assumes that a very small amount of spread (micrometastatic disease) has already happened. (micrometastatic disease) has already happened. The main treatments are The main treatments are chemotherapy, surgery, and chemotherapy, surgery, and radiationradiation. Both surgery and radiation are effective . Both surgery and radiation are effective treatments for the primary tumor.treatments for the primary tumor. Many surgeons remove the tumor when doing so will Many surgeons remove the tumor when doing so will not cause a major loss of function of the body part not cause a major loss of function of the body part involved. Many doctors use radiation treatment only involved. Many doctors use radiation treatment only when a tumor cannot be removed completely or when a tumor cannot be removed completely or without the patient losing function.without the patient losing function.

CHEMOTHERAPYCHEMOTHERAPYChemotherapy is used to kill the primary tumor and Chemotherapy is used to kill the primary tumor and any unknown microscopic spread. any unknown microscopic spread. Chemotherapy uses multiple medications. The most Chemotherapy uses multiple medications. The most common agents are common agents are vincristine , dactinomycin , vincristine , dactinomycin , cyclophosphamide, and doxorubicincyclophosphamide, and doxorubicin. Recent evidence . Recent evidence shows the addition of ifosfamide and etoposide helps. shows the addition of ifosfamide and etoposide helps. Chemotherapy is given through an indwelling central Chemotherapy is given through an indwelling central venous catheter. This is placed just before treatment venous catheter. This is placed just before treatment is started. is started. Chemotherapy is done in cycles. It uses combinations Chemotherapy is done in cycles. It uses combinations of the various drugs. The time between cycles lets the of the various drugs. The time between cycles lets the blood cell count recover. This includes white blood blood cell count recover. This includes white blood cells and platelets. The drugs depress them. The cells and platelets. The drugs depress them. The patient will lose their hair. patient will lose their hair. It generally takes one year to finish all the cycles of It generally takes one year to finish all the cycles of chemotherapy, with surgery and/or radiation. chemotherapy, with surgery and/or radiation.

SURGERYSURGERY

Surgery is used to remove the primary tumor and Surgery is used to remove the primary tumor and occasionally metastatic lesions. occasionally metastatic lesions. The main goal of a tumor surgery is to remove the tumor The main goal of a tumor surgery is to remove the tumor entirely and keep it from returning; the reconstructive entirely and keep it from returning; the reconstructive issues, while important, are nonetheless secondary goal. issues, while important, are nonetheless secondary goal. In many cases, doctors can avoid amputation by surgically In many cases, doctors can avoid amputation by surgically removing the primary tumor. Rebuilding the bone and removing the primary tumor. Rebuilding the bone and joints is then needed. Infections and problems with joints is then needed. Infections and problems with prosthesis and wound healing are the most frequent prosthesis and wound healing are the most frequent concerns. concerns.

RADIATION THERAPYRADIATION THERAPY

Radiation is used to lower the chance of recurrence. Radiation Radiation is used to lower the chance of recurrence. Radiation may be used instead of surgery at sites where surgery is too may be used instead of surgery at sites where surgery is too risky or complicated. risky or complicated. When radiation treatment is used, daily treatments are given When radiation treatment is used, daily treatments are given over the course of many weeks.The risks associated with the over the course of many weeks.The risks associated with the radiation include: radiation include:

Skin damage Skin damage Muscle scarring and loss of joint flexibility Muscle scarring and loss of joint flexibility Damage to nearby organs Damage to nearby organs Loss of bone growth in growing children Loss of bone growth in growing children Secondary cancers caused by radiation Secondary cancers caused by radiation

PROGNOSISPROGNOSISThe outcome for patients with Ewing's sarcoma The outcome for patients with Ewing's sarcoma has improved considerably due to improvements has improved considerably due to improvements in chemotherapy, diagnostic imaging, and in chemotherapy, diagnostic imaging, and reconstructive techniques. reconstructive techniques. In general, two thirds of patients without any In general, two thirds of patients without any demonstrable spread will survive at least five demonstrable spread will survive at least five years after diagnosis with standard treatment. years after diagnosis with standard treatment. When tumors come back, it usually happens When tumors come back, it usually happens within the first few years after treatment. within the first few years after treatment. The absence of known spread is the most The absence of known spread is the most important factor in favor of being more likely to important factor in favor of being more likely to survive. Other factors include having excellent survive. Other factors include having excellent response to chemotherapy, primary tumors in response to chemotherapy, primary tumors in the extremities instead of pelvis, and complete the extremities instead of pelvis, and complete removal of the tumor. removal of the tumor.