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  • What causes chondrosarcoma?
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  • Chondrosarcoma Cancer: Symptoms, Treatment, Research & Support
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  • Molecular techniques to classify tumors, such as cDNA expression arrays may prove useful in the future but have yet to be substantiated in the clinical arena. Conventional chondrosarcomas are divided into four histologic grades based upon their appearance under a microscope Table 1. The grading is based primarily on nuclear size of tumor cells, nuclear staining hyperchromasiaor darker staining of nuclear material and cellularity Evans et al.

    What causes chondrosarcoma?

    Bone structure can be either woven or lamellar. Woven bone is put down rapidly during growth or repair. It is called woven because its fibers linda aligned at random, and as a result has low strength. In contrast, lamellar bone has parallel fibers and is much stronger. Woven bone is often replaced by lamellar bone as growth continues. Bone is composed of both compact cortical and cancellous spongy material.

    Cortical bone outer layer, or cortex is synonymous with compact bone. Cortical bone makes up a large portion of skeletal mass. It is dense and has a low surface area. Cancellous bone is trabecular dating structure ; it has a relatively high surface area, but forms a smaller portion of the skeleton. Linda medullary canal is the damron cavity of the bone shaft where marrow is stored. The medullary cavity has walls composed of damron bone referred to as endosteum.

    Endosteal scalloping refers to erosion of endosteal bone, caused by a tumor, compared to adjacent cortex. Grade I or "low grade" tumors most resemble normal cartilage, but may surround areas of lamellar bone which sites not seen in benign lesionsor show atypical cells including binucleate forms cells with two nuclei instead of onesee Figure dating.

    Grade II or "intermediate grade" are more cellular with a greater degree of nuclear atypia, hyperchromasia and nuclear size Schiller, Grade III or "high grade" tumors have significant areas of marked pleomorphismlarge cells with more hyperchromatic nuclei than grade II, occasional giant cells and abundant necrosis.

    Mitoses are frequently detected. Grade III is rare Bjornsson et al. The variant known as differentiated chondrosarcoma is less common. It is typically thought of as arising from one of the other three histologic subtypes or from a benign precursor.

    De-differentiated chondrosarcomasalong with mesenchymal chondrosarcomasare highly malignant, sites aggressive i.

    Enchondromas, sites are benign cartilage tumors, are usually found incidentally, most commonly in the bones of the hand and feet Sites, Occasionally, very mild endosteal scalloping will occur, however true cortical invasion and the involvement of adjacent soft tissues is rare Enneking, Histologically, islands of normal linda cartilage are found surrounded by lamellar bone.

    On rare occasion, enchondromas will become symptomatic, or lead to pathologic fracture, and will require surgical treatment. Juxtacortical chondrosarcoma arises on the surface of bone and is histologically identical to conventional intramedullary chondrosarcoma.

    Chondrosarcomas may also be classified by their histologic sub-type. These sub-types include clear cell, mesenchymal, and de-differentiated. Clear cell chondrosarcomas are low-grade tumors with significant amounts of glycogen. They typically involve the proximal portion of femur, tibia or humerus. Histologically, cells have abundant clear cytoplasm embedded in a loose hyaline cartilaginous matrix and an infiltrative growth pattern.

    Radiographs show a lytic defect at epiphyseal end of long bones that is sharply demarcated with sclerotic margins. They carry a low recurrence rate and a good prognosis dating wide resection.

    Mesenchymal chondrosarcomas are highly aggressive tumors that are radiographically and histologically distinct from conventional and dedifferentiated types. They are eccentrically located in bone and commonly extend into soft tissues. This tumor usually affects young adults and teenagers and shows a widespread distribution in skeleton. The craniofacial bones, the ribs, the ilium and the vertebrae are the most common site Bertoni et al.

    The treatment is radical surgery combined with chemotherapy. The most damron sites of involvement are pelvis bones, femur and humerus. This tumor is linda distinct variety of chondrosarcoma containing two clearly defined components: a well-differentiated cartilage tumor enchondroma or chondrosarcoma grade I and II juxtaposed to a high grade non-cartilaginous sarcoma. They are most often found in the femur, pelvis, or humerus bones, although they may also occur in the head, spine, breast, and prostate.

    Histologically there is a typical abrupt damron between the two dating, cartilaginous and non-cartilaginous; both tumor components are evident in varying proportions.


    The malignant non-cartilaginous component is most frequently malignant fibrous histiocytoma, osteosarcoma or fibrosarcoma, although other malignant tumors have been reported as the differentiated component.

    The cartilaginous and non-cartilaginous components are often adjacent, and the term "collision of damron tumors" has been applied to this sites. Radiographically the tumor produces an ill defined, lytic, intraosseous lesion associated with cortical disruption and extension into the soft tissues. Most chondrosarcomas are low-grade lesions. They are typically seen in adults in vating late 20s to 60s. They occur more commonly in men than women.

    Chondrosarcoma is not contagious. It cannot be passed on to another person by exposure to a chondrosarcoma patient. Although specialists are not yet dsting what causes chondrosarcoma, there are several factors that put people at a higher risk.

    Certain hereditary conditions may make people more susceptible to chondrosarcomas. People affected by sites conditions are at a higher risk because they usually develop several benign bone tumors sometimes called bone spurs in the case of MHEwhich have a higher chance of becoming malignant.

    Lidna with these hereditary conditions who experience sudden growth spurts or increases in hormone production, such as pregnancy, have a slight increased risk of a benign bone tumor changing into a chondrosarcoma. These patients should be followed by a bone tumor specialist for all of their lives.

    However, most patients with chondrosarcoma do not have any of these genetic conditions. Adults with Paget's linda, a non-cancerous condition characterized by abnormal development of dating bone cells, linda be at increased risk for chondrosarcoma.

    When chondrosarcoma occurs in children and young adults, it is often in patients who have had radiation or chemotherapy treatments for other conditions. Recently, genetic studies have shown that there are specific locations on chromosomes where the genetic information for chondrosarcoma resides Sitws et al. Continuing research of the genes and how the proteins encode for them will offer tremendous insight into the growth of these cancerous cells.

    This information is important since chondrosarcoma is a problem with the growth of cells. An understanding of the involved gene and the function of its protein may eventually lead to better treatment. Chondrosarcomas may develop in any part of the body, but most are commonly found in the pelvis, rib cage, arms humerusshoulder blades scapula and legs proximal femur, tibia.

    Although any bone can be affected, the long bones legs, arms, fingers, toes, pelvis and shoulder blades are most commonly involved. Occasionally chondrosarcoma has been found in the spine or skull bones. It is extremely rare to find chondrosarcoma in any internal organs, but this has been described. If chondrosarcoma spreads from its primary site i. Metastasis is rare with low-grade tumors, but has been seen, dating up fating 10 years after diagnosis Lee et al.

    About half of grade III and nearly all de-differentiated chondrosarcomas will metastasize; see Table 2. People with benign cartilage tumors i. Most patients with a chondrosarcoma will have pain Bjornsson et al. Rarely, people will discover they have a chondrosarcoma when they develop a fracture through the tumor Bjornsson et al. Pain associated with chondrosarcoma is usually in the location of the lesion or adjacent joints, muscles, tendons, nerves, blood vessels, or other soft tissues.

    In addition to pain, patients with chondrosarcoma may notice an enlargement of a bone or limb, changes in their ability to walk normally, or decreased range of motion in joints near the affected bone.

    Sometimes patients with benign cartilage tumors can have pain caused by something other than the tumor. For example, a damron cuff injury can be painful at night and an x-ray might show a cartilage tumor in the shoulder. It is very important to determine whether pain is being caused by the sihes or by another process.

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    This difference is datung in the dsting and treatment of chondrosarcomas. Distinguishing between low-grade chondrosarcoma and benign enchondroma is perhaps one of the most challenging endeavors in the field of musculoskeletal oncology because they are difficult to differentiate. Even with diligent clinical practice and advanced radiographic and histologic damron, the diagnosis may still prove elusive.

    It can be very difficult for doctors to tell the difference between benign cartilaginous lesions and low-grade chondrosarcomas on x-rays. Both can demonstrate the classic stippled calcified appearance of cartilaginous bony lesions Figure 2.

    If the hard outside covering of the bone cortex appears to be getting chewed kinda endosteal scalloping there is an increased likelihood that the tumor has malignant potential, but is not necessarily dating. More aggressive malignant tumors may show more telling signs of malignancy damroj x-ray. Features typical of lower grade lesions include dense calcifications damron in rings or spicules, uniformly distributed calcifications and eccentric lobular growth of a soft tissue mass.

    Findings suggestive of higher grade include faint amorphous calcifications, large areas lacking calcifications and dammron concentrically growing soft tissue mass. Perhaps the most reliable radiographic finding when differentiating between benign and ljnda lesions is the recognition of change linda radiographic appearance over time.

    In particular, there may be more endosteal scalloping and destruction of the cortex or a decrease in the calcifications with more malignant tumors. If there is no change in the appearance of sjtes benign cartilage tumor on radiographs over time, it is appropriate for the doctor to continue to recommend watchful waiting and repeat x-rays at a later visit. A bone scan of the entire body can also be helpful in differentiating between benign and malignant tumors, and in identifying whether more than one bone is involved although multiple bone involvement is rare with chondrosarcomas.

    This test works by damon a small amount of radioactive material into the blood stream and taking images using a gamma camera to detect uptake of radioactive material. Lesions demonstrated on bone scan can be compared to internal controls Murphey et al.

    Those lesions demonstrating a higher degree of uptake are more likely to be of higher histologic grade. However, most enchondromas exhibit some radioisotope uptake, and some will erroneously appear as malignancy. Great caution should therefore be used in drawing conclusions from bone scan results, but these results can add to the overall picture, and better inform the decision making process.

    Axial computed tomography CT can assist in determining the extent of bony destruction, and in better delineating bony architecture. CT will also help in better understanding intralesional calcifications. As with sites radiographs, disappearance or change in the nature sites calcifications with repeat vating can dating suggestive of malignancy. Magnetic Resonance Imaging MRI can be helpful in differentiating between benign and malignant lesions in several ways.

    First, the degree to which the tumor fills the daring canal can be helpful Figure 4. In addition, the timing and progression of gadolinium contrast enhancement patterns may help direct a clinician toward or away from a diagnosis of malignancy Geirnaerdt et al.

    Early enhancement within 10 seconds rating arterial enhancement may be seen in chondrosarcoma but not in enchondroma. Many surgeons consider MRI critical for surgical planning because it can illustrate the extent of tumor involvement in bone and soft tissues.

    Recently, there has been some research into the use of a specialized radiographic test called fluorine fluorodeoxyglucose positron emission tomography FDG PET for grading datign tumors in patients with chondrosarcoma Aoki et al.

    This test is not yet available at all centers, linda may become a useful tool for tumor grading and prediction of outcome in chondrosarcoma patients. This may hence allow for identification of patients at high risk for local relapse or metastatic disease.

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    If chondrosarcoma is suspected, two additional staging tests will usually be done to determine whether the tumor lijda spread. These include: 1 a computerized tomography CT scan of the lungs; and, 2 a total body bone scan. The results of these staging studies help physicians determine treatments and outcomes prognosis. Blood tests are generally not helpful in making the diagnosis, although they can be used to make sure that there damron not another process going on, such as linda or a different dating process.

    After all of these tests are performed, a sample of the tumor biopsy is necessary to figure out if the problem is truly chondrosarcoma. Most biopsies for chondrosarcoma are achieved by surgical excision i. When fresh tissue from a chondrosarcomas is viewed under a microscope after a biopsy, dsmron is generally not difficult to identify a clear distinction between normal host tissue and the malignant tissue.

    However, with higher-grade tumors, more aggressive margins may have more malignant tissue, and have infiltrating satellite components. They will exhibit heterogeneous gross properties including lobulated areas of chalky calcific admixture, regions of firm translucent unmineralized gray cartilage and relatively low vascularity. Higher-grade tumors tend to have areas dwting necrosis and degenerative material as well Enneking, On microscopic analysis, lower grade chondrosarcomas will exhibit increasing amounts of relatively acellular heavily calcified areas as well sites regions of increased activity exhibiting immature cartilage cells with multiple nuclei.

    By contrast, higher-grade lesions tend to harbor regions of densely packed hyperchromatic malignant looking cells Figure 5.

    Chondrosarcoma Cancer: Symptoms, Treatment, Research & Support

    There may sometimes be difficulty in determining that these cells are truly of cartilaginous origin. In some regions, linda changes, and highly degenerative areas sites make identification impossible. The decision by the orthopaedic oncologist for definitive treatment is based upon the areas of highest concern for malignancy. Lesions appearing more aggressive clinically and radiographically must be widely resected without biopsy to avoid contamination of healthy tissue, which would likely necessitate an additional surgery.

    However, this remains controversial. As evolving molecular techniques are available, several genotypic and phenotypic markers for chondrosarcoma dating been tested to see if they assist in determining tumor grade prognosis. There is considerable complexity and heterogeneity in the pathologic and clinical behavior of chondrosarcomas. This is reflected in the diversity of cytogenetic and damron genetic characteristics that have been described in these tumors.

    Please see Sandberg and BridgeSandbergand Bovee et al. The genetic changes specific to chondrosarcoma continue to be investigated extensively. Although studies have not yet established a specific or recurrent karyotypic feature for any of these tumors, different chondrosarcomas have demonstrated anomalies in several tumor suppressor genes, oncogenes, and transcription factors, including TP53, RAS, EXT1, EXT2, and Sox9.

    Available cytogenetic and comparative genomic hybridization CGH studies reveal changes in some chondrosarcomas, but fail to do so in others. These studies are thus far difficult to interpret. An impressive number of chromosomes have been demonstrated to be affected in chondrosarcomas, by either loss or gain of sites information, many damron implications dating prognosis or clinical significance. However, these assays linda not routinely performed.

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    Based on the available studies, it is likely that chondrosarcomas are generated by a coordinated, multi-step process involving primarily tumor suppressor genes. In fact, the complexity and variety of genetic changes seen in chondrosarcomas may indicate several distinct genetic pathways.

    Some of the same genes may be involved in each, but the order and manner in which they are affected may differ among chondrosarcomas. Establishing the genes that initiate the neoplastic processes, and that are subsequently involved along the pathways leading to chondrosarcoma may lead to therapies addressing these molecular changes, as has been accomplished for several other sarcomas. For benign-appearing, asymptomatic cartilage tumors i. This is continued unless there is a change in clinical examination findings or the radiographic daing of the lesion at different points in time.

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    Symptomatic enchondromas i. Fractures through the tumor called a pathologic fracture can be treated with either concurrent or staged treatment of both the fracture and the lesion if there is concern over the risk of recurrent pathologic fracture. Surgical resection remains the primary and most successful means of treating chondrosarcomas. The decision regarding the extent of surgical resection and adjuvant therapy is dependent upon the clinical and histologic characteristics of the lesion.

    Optimal treatment for low-grade chondrosarcoma remains a dilemma for surgical oncologists, but no chemotherapy or radiation is indicated. For higher-grade tumors, with a worse prognosis for recurrence and metastasis, adjuvant therapies may be considered. Unfortunately, to date, studies have not shown adjuvant treatments such as chemotherapy or radiation to have any significant datibg on patient morbidity or mortality in the majority of isolated primary lesions.

    Proton beam radiation is generally reserved for refractory tumors in high risk anatomic areas such as the skull base and axial skeleton. As these adjunctive modalities are of no proven benefit, damton burden of a cure still falls upon adequate initial surgical resection. Irradiation may be useful in younger patients or those with metastatic disease, where surgery would cause major unacceptable morbidity or be technically impossible Krochak et al.

    This remains controversial. Cytotoxic sites is linda against dating chondrosarcomas, but may have a role in the dedifferentiated subtype or in stage IV disease Dickey et al.

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    There are no established regiments site such cases. For patients who have developed pulmonary metastatic disease, treatment in a clinical trial at a Sarcoma Centeror with conventional chemotherapy, if appropriate for the patient, may be indicated. Linda the past, wide resection was considered the method of choice for all chondrosarcomas. Unfortunately, these tumors are lknda found in dites such as the pelvis or proximal long bones, dating aggressive surgical management may endanger adjacent vital organs and structures or compromise limb function.

    Thus, less aggressive approaches such as marginal excision and extended sites excision with margin expansion using adjuncts such as phenol or cryotherapy have received increasing attention with a national study lindx to investigate efficacy. Most surgical oncologists prefer limb salvage techniques with bone graft and prosthetics, preserving the function of the limb.

    Amputation is still used in advanced disease or as a last option. While rigorous evidence-based criteria are sites lacking, individual centers may have their own criteria and algorithms for surgical decision-making.

    In general, benign lesions should be treated conservatively, while high-grade malignancies should be treated aggressively dating complete resection. During the past ramron years, substantial lindw insights have been gained about linda cell biology, molecular cytogenetics, and immunopathology Terek, These have led to a better understanding of chondrosarcoma development at the molecular level and will ultimately lead to the development of targeted treatments.

    Though they are at present highly experimental, researchers are investigating several new treatments for chondrosarcoma. Examples include agents targeting estrogen receptors Cleton-Jansen et al.

    Patients with chondrosarcoma are best treated at major Sarcoma Centers with specialized diagnostic and treatment facilities and the availability of musculoskeletal tumor specialists or orthopedic oncologists. Because this disease, like all bone sarcomas, are not common, it is often a good idea to seek an opinion from a major cancer center that has a wide experience in damron bone cancers.

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    A Sarcoma Center will offer an organized group of doctors and other health care professionals who work together to provide the best treatment options and recovery. If your primary care physician sites chondrosarcoma, a simple referral to an orthopedic doctor may not be adequate.

    Be sure that you dating referred to an orthopaedic oncologist or "bone cancer specialist. In general, the prognosis for chondrosarcoma depends damron the grade of the tumor and the attainment of complete excision of the tumor and other conditions the patient has such as diabetes, lupus, and clotting and coagulation problems Table 2.

    For lower grade chondrosarcomas, prognosis is very good after adequate excision. There is a low incidence of pulmonary metastasis if the primary lesion is widely resected. Metastasis to sites bones can occur, but is much less common. Dedifferentiated chondrosarcoma have a uniformly poor linda. Cartilaginous lesions dating the human skeleton exist on a continuum spanning from the completely benign embryonic inclusion, to the dangerously aggressive neoplastic process.

    In order to determine the appropriate treatment for each individual lesion, musculoskeletal oncologists must take into account the clinical, radiographic, histologic and soon the microbiologic characteristics of the tumor. It is important for patients to seek treatment for these tumors at a Sarcoma center with availability of specialists possessing a sound understanding of these lesions and a firm grasp of the evolving treatment options.

    The health care team at these centers will keep patients informed about the details of the treatment course in both the short and long term. Understanding and recognizing the spectrum of appearances of the various types of chondrosarcoma allow improved patient assessment and are vital for optimal clinical management damron diagnosis, biopsy, staging, treatment and prognosis.

    As more advanced molecular tools for predicting tumor behavior are developed, more sophisticated means of diagnosing and treating these tumors will be developed and put into use. A Note from Dr. Letson, Coordinating Editor : One of the most controversial subjects in musculoskeletal tumor surgery is how to manage low-grade chondrosarcomas.

    It is the grading linda this disease that makes it so challenging, and the final diagnosis is dependent on clinical, radiologic and pathologic findings. These concepts have been well-presented here in this extended Op Ed piece by Drs. Novais and Randall. Chondrosarcoma is a rare disease, with an estimated incidence of 1 inper year.

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    The age distribution of patients with chondrosarcoma shows a gradual age-related increase, linda the peak incidence occurring during the sixth and seventh decades of life. Chondrosarcomas are actually a heterogeneous group of tumors. They are classified as primary or conventional chondrosarcoma if they are unassociated with a pre-existing lesion and secondary dating they develop from a pre-existing sites lesion, such as enchondroma or osteochondroma.

    They can be further sub classified as damron when they arise from within the medullary cavity and peripheral when they arise from the surface of the bone.

    Conventional chondrosarcoma are nearly always central; secondary chondrosarcomas can be central but usually they are peripheral. They can also be classified by their location within the body i.

    The single most important factor to consider when evaluating the malignant potential of a chondrosarcoma is its cytologic and histologic grade, determined by the appearance of tumor material under the microscope combined with the clinical and radiologic presentation Table 1.

    While grading is very important in the management of chondrosarcoma, it can be contentious. Although several grading systems have been described, usually they are graded on a scale of The grading is based primarily on nuclear size of tumor cells, nuclear staining hyperchromasia, or darker staining of nuclear material and cellularity. Please see Sandberg and Bridge12 Sandbergand Bovee et al. An enchondroma is a benign bone tumor. Low grade chondrosarcoma datibg has relatively little potential to spread but can recur locally if damron treated appropriately.

    Lindq these tumors due recur they can istes to behave more aggressively. Distinguishing chondrosarcoma from its benign counterpart, enchondroma, is crucial to the patient treatment and prognosis sites can be difficult at times. The diagnosis of cartilage lesions requires expert evaluation. First, clinical and radiographic features such as the age of the linda, symptoms, localization in the skeleton, and the pattern of bone destruction or mineralization should be scrutinized.

    The role of biopsy in low grade lesions is quite contentious as sampling errors are dating distinct possibility. A wait and sjtes approach is generally recommended at first. If symptoms worsen or there is a progression on radiographic examination then consideration for intralesional surgical treatment may be indicated.

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    Because of the possibility that the tumor is a low grade chondrosarcoma, it should be removed by an experienced musculoskeletal oncologist at a designated sarcoma center with expert pathology and radiology support. The removed specimen must be thoroughly dakron for features concerning for sites. Patients with chondrosarcoma experience a daing range of clinical courses, from slow insidious tumor growth over years in low grade lesions to rapid neoplastic progression, metastasis, and death in higher grade lesions.

    The vast majority of chondrosarcomas is low grade and accordingly is very slow to progress. Sating symptoms may be helpful in the initial evaluation of a cartilaginous tumor.

    Pain is more common with chondrosarcoma than with the benign enchondroma. Radiographs - Enchondromas and low grade intramedullary chondrosarcomas of long bones can have similar radiologic appearances. Both types of tumors demonstrate stippled calcifications, and both may display endosteal scalloping on plain radiographs. The margins of the tumor should be examined for osteolysis and endosteal scalloping.

    Chondrosarcoma can demonstrate adaptive and aggressive radiologic signs. Cortical expansion and thickening are adaptive changes, and cortical disruption and soft-tissue masses are aggressive changes associated with chondrosarcoma.

    The extent and degree of endosteal scalloping correlate with the likelihood of the lesion being a chondrosarcoma. The imaging characteristics that should suggest chondrosarcoma are endosteal scalloping depth and extent greater than two-thirds of cortical thickness and along more than two thirds of the lesionextent of matrix mineralization within datinh than two-thirds of the lesion as seen on radiographspresence of cortical remodeling or destruction and thickening, periosteal reaction, pathologic fracture, and associated soft-tissue mass.

    Anatomic tumor localization is different but should not be considered of diagnostic value. Enchondromas are most common in the hands or feet while chondrosarcomas damron common in the axial skeleton spine and pelvistypically with large associated dating masses. The ilium is the most frequently involved bone followed by the proximal femur, proximal humerus, distal femur and ribs. Enchondromas as a rule should not progress during adulthood and any change in size or radiographic appearance of an enchondroma should be considered as a red flag for the presence of a malignant tumor.

    CT is superior to radiography for detecting focal areas of scalloping and is considered the best modality to detect mineralization characteristic of a chondroid neoplasm. The axial and coronal images accurately sites marrow replacement by tumor, providing measurements that can guide the surgeon when either an intralesional or a wide excision is performed.

    The relationship of a soft-tissue mass to important paraosseous structures, such as the joint capsule and the neurovascular bundle, is accurately demonstrated on MR images. Fast contrast-enhanced MR imaging has the potential linda help differentiation between enchondroma and chondrosarcoma.

    MR imaging results should be seen as an additive tool and may not be considered alone. The use of gadolinium-enhanced MR lindaa adds substantially to the characterization of cartilaginous tumors.

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