eMedicine Specialties > Radiology > Musculoskeletal
Chondrosarcoma
Updated: Nov 3, 2009
Introduction
Background
Chondrosarcoma is the second most frequent primary malignant tumor of bone, representing approximately 25% of all primary osseous neoplasms. Chondrosarcomas are a group of tumors with highly diverse features and behavior patterns, ranging from slow-growing non-metastasizing lesions to highly aggressive metastasizing sarcomas.1,2,3
Frontal radiograph of the left fibula head demonstrates a lucent lesion that contains the typical chondroid matrix calcification. Low-grade tumor.
Giuffrida et al used the Surveillance, Epidemiology and End Results (SEER) database to identify demographic and prognostic characteristics of chondrosarcoma and to describe its natural history following treatment. Multivariate analysis of the 2,890 cases of chondrosarcoma in the United States over the last 30 years showed that survival rates did not change over that period and that only grade and stage are independent prognostic factors for survival. Giuffrida et al recommended that routine post-treatment surveillance be extended to 10 years of follow-up; beyond that point, patients were more likely to die of causes unrelated to chondrosarcoma.4
Pathophysiology
Chondrosarcoma is a malignant tumor of cartilaginous origin in which tumor matrix formation is entirely chondroid in nature.
Chondrosarcomas are classified as central (originating within the intramedullary canal) or peripheral. Rarely, they arise as juxtacortical lesions. Lesions are designated as primary when they arise de novo or as secondary when they occur within a preexisting lesion such as an enchondroma or osteochondroma.
Tumors are further categorized by grade. Grade 1 represents the least aggressive in terms of histologic features, and grade 3 represents the most aggressive. Most chondrosarcomas are pathologically classified as conventional; other subgroups are clear cell, myxoid, mesenchymal, and dedifferentiated. This article deals exclusively with conventional chondrosarcomas of the bone.
Frequency
United States
The incidence rate of chondrosarcoma is dependent on patient age; the incidence peaks at 8 cases per 1 million population in those 80-84 years of age. The incidence in children is low. Most tumors arise in patients older than 40 years. The risk of chondrosarcoma is increased in people with enchondromatosis syndromes (eg, Ollier disease, Maffucci syndrome, metachondromatosis) and in those with hereditary multiple exostosis (eg, diaphyseal aclasis). Patients with these conditions are generally younger than other patients at the time of presentation.
Mortality/Morbidity
For patients with chondrosarcoma, the overall prognosis is related to the size of the lesion, its anatomic location, and its histologic grade.
- Patients with axial lesions have a worse prognosis than those with lesions of the appendicular skeleton.
- The 5-year survival rate for patients with grade 1 lesions is 90%; the rate decreases to 29% for patients with grade 3 tumors. Grade 1 lesions do not metastasize. Metastatic spread, typically pulmonary, is more frequently associated with grade 3 lesions than with other grades of lesions. Lymph node spread is more common with chondrosarcoma than with other osseous neoplasms.
- Tumor recurrence typically occurs 5-10 years after surgery. Recurrent chondrosarcoma is often more aggressive than the original lesion, and the histologic grade is often higher.
Race
No major difference in incidence is observed between ethnic groups.
Sex
A slight male predilection exists, with a male-to-female ratio of 1.5-2:1.
Age
The age range is wide; most cases occur in patients older than 40 years.
- Secondary chondrosarcomas tend to occur in patients 20-40 years of age.
- Chondrosarcoma is rare in children; when it does occur, it tends to be aggressive.
Anatomy
Tumors are predominantly axial; they most commonly involve the pelvic bones, femur, humerus, ribs, scapula, sternum, or spine. In tubular bones, the metaphysis is the most common site of origin. The proximal metaphysis is more frequently involved than the distal end of the bone. Involvement of the distal humerus is most unusual. Chondrosarcoma rarely occurs in the hands and feet; such occurrences usually arise as a complication of a multiple enchondromatosis syndrome. Chondrosarcoma arising de novo in the hands and feet is extremely unusual.
Presentation
The most common symptom at presentation is pain, which is often present for months and typically dull in character. It may be worse at night. Local swelling may be present. When the tumor occurs close to a joint, effusion may be present, or movement may be restricted. The average duration of symptoms before presentation is 1-2 years. The tumor may occasionally occur as a pathologic fracture.5,6,7,8
Preferred Examination
Radiographs are essential for the initial diagnosis of chondrosarcoma; radiography is sometimes supplemented with CT, which is more sensitive for detecting matrix calcification and for confirming deep endosteal cortical scalloping in intramedullary tumors.
MRI is the preferred modality for evaluating the extent of intramedullary tumors and for demonstrating extraosseous extension. MRI is useful in evaluating the thickened cartilage cap in an osteochondroma in which a secondary chondrosarcoma develops. MRI is less sensitive than CT in identifying small amounts of matrix calcification within a tumor.
The imaging appearances of chondrosarcoma may overlap with those of other lesions, especially other cartilaginous tumors such as enchondroma. The presence of pain with any lesion (without a pathologic fracture in lesions of the hands and feet) is highly suggestive of malignancy. Other findings suggestive of malignancy in a cartilaginous tumor include endosteal cortical scalloping of more than two thirds of the thickness of the cortex, ill-defined border and/or zone of transition, and a large soft tissue mass. Both benign and malignant cartilaginous tumors may show central lucency; however, lucency of an area that previously showed matrix calcification is highly suggestive of chondrosarcoma.
Limitations of Techniques
See Preferred Examination, above.
Differential Diagnoses
Bone Infarct
Enchondroma and Enchondromatosis
Osteochondroma and Osteochondromatosis
Osteosarcoma, Classic
Osteosarcoma, Variants
Other Problems to Be Considered
Central
Enchondroma
Osteosarcoma
Fibrosarcoma
Bone infarct
Osteochondroma
Parosteal osteosarcoma
Radiography
Frontal radiograph of the left fibula head demonstrates a lucent lesion that contains the typical chondroid matrix calcification. Low-grade tumor.
Frontal radiograph of the left acetabulum demonstrates an expansile lucent lesion with no internal matrix calcification. Low-grade central tumor.
Frontal radiograph of right side of upper abdomen demonstrates a destructive, expansile lesion of the 12th rib. The lesion contains irregular calcification. High-grade central tumor.
Frontal radiograph of the pelvis demonstrates extensive calcification overlying the left ilium and in the lateral soft tissues. No bone destruction is shown. High-grade secondary peripheral tumor.
Lateral radiograph of the distal femur in a patient with hereditary multiple exostoses. Several osteochondromas of varying appearances arise from the metaphyseal region; these typically grow away from the joint. Soft tissue calcification is shown overlying the most posterior osteochondroma. High-grade secondary peripheral tumor.
Findings
Radiographs typically show a lucent lesion, which frequently contains matrix calcification, particularly in well-differentiated tumors. The degree of organization of the matrix calcification may be correlated with the grade of the tumor. Aggressive tumors contain irregular calcifications, and they often have large areas showing no calcification at all. Well-differentiated lesions tend to have more developed matrix; the typical appearance is of rings and arcs (see Images above).
The margin of intramedullary lesions is determined by the degree of aggression of the tumor. It is frequently ill defined. Endosteal scalloping may be present; when its depth is more than two thirds the normal thickness of the cortex, this scalloping is useful in distinguishing chondrosarcoma from enchondroma, except in lesions of the hands and feet. Benign enchondromas in these areas may cause considerable cortical thinning; such enchondromas may occur as a pathologic fracture.
The presence of cortical destruction or a soft tissue mass is indicative of malignancy. Destruction of matrix calcification that was previously visible in an enchondroma is indicative of malignant transformation.
Degree of Confidence
Radiographs alone are often inadequate for assessing the size of the tumor; MRI is useful for demonstrating both the intramedullary extension and the soft tissue extension of the lesion. CT may be helpful in identifying matrix calcification in some lesions that appear entirely lucent on radiographs.
Computed Tomography
Bone-window CT scan of left acetabulum demonstrates matrix calcification in the expansile lucent lesion in the anterior column (see also Images 2, 4, and 5 in Multimedia Section). Low-grade central tumor.
CT of the right side of the upper abdomen demonstrates the expansile tumor with a large associated soft tissue mass containing foci of calcification (see also Image 6 in Multimedia Section). High-grade central tumor.
CT scan of the pelvis demonstrates a large soft tissue mass that contains calcification arising from a broad-based sessile osteochondroma on the posterior aspect of the ilium (see also Images 8 and 10 in Multimedia Section). High-grade secondary peripheral tumor.
CT scan of the distal femur demonstrates a broad-based osteochondroma with a thick overlying soft tissue cap that contains focal calcification. The metaphyseal contour is irregular because of the presence of several other osteochondromas in this patient with hereditary multiple exostoses (see also Images 11 and 13 in Multimedia Section). High-grade secondary peripheral tumor.
Findings
In as many as 90% of cases, tumors appear as lucent areas containing chondroid matrix calcification. Endosteal scalloping and cortical destruction are frequently easier to appreciate on CT scans than on radiographs (see Images above).9
CT may be used to guide percutaneous biopsy, and it is the modality of choice for investigating possible pulmonary metastatic disease.
Degree of Confidence
CT may often be used to successfully categorize the lesion as being of cartilaginous origin. The medullary extension of the lesion may be assessed more accurately with CT than with radiography. However, MRI is superior, and it is also the most useful modality for determining soft tissue extension.
Magnetic Resonance Imaging
T2-weighted axial MRI of the pelvis demonstrates the high signal intensity of the acetabular lesion (same patient as in Images 2-3, 5 in Multimedia). Low-grade central tumor.
T1-weighted axial MRI of the pelvis demonstrates the low signal intensity of the acetabular lesion (same patient as in Images 2-4 in Multimedia). Low-grade central tumor.
T2-weighted axial MRI of the pelvis demonstrates a lobulated high-signal-intensity soft tissue with local-signal-intensity septa arising from the osteochondroma on the posterior aspect of the ilium (same patient as in Image 9 in Multimedia). Several areas of low signal intensity are shown; these correspond to focal areas of dense calcification. This appearance is typical of cartilaginous material. High-grade secondary peripheral tumor.
Fast spin-echo T2-weighted axial MRI of the distal femur in a patient with hereditary multiple exostoses (same patient as in Images 11-12 in Multimedia). Image demonstrates the thick cartilage cap overlying a broad-based osteochondroma. Areas of focal reduced signal intensity in the cartilage cap correspond to foci of dense calcification. High-grade secondary peripheral tumor.
Findings
MRI typically demonstrates lobulated lesions of high signal intensity on T2-weighted images. Lobules are commonly separated by septa of low signal intensity. On T1-weighted images, the lesion generally displays low signal intensity (see Images above).10
Areas of matrix calcification are shown as signal voids on images obtained with all sequences, but small amounts of calcification may not be identifiable. MRI may demonstrate large aggregates of calcium, but tiny scattered calcifications may be completely missed because of partial-volume averaging. MRI may be used to assess soft tissue extension and the intramedullary extent of the tumor. MRIs may demonstrate endosteal cortical scalloping, but this feature is more easily assessed with CT.
MRI is useful in assessing the thickness of the cartilage cap of osteochondromas to identify chondrosarcoma transformation. Chondrosarcomas show variable patterns of enhancement after the administration of contrast material.
Degree of Confidence
MRI is the method of choice for clarifying the intramedullary and extraosseous extension of a chondrosarcoma; features related to cortical bone and matrix calcification are more accurately assessed with CT.
Ultrasonography
Findings
Ultrasonography has no role in the evaluation of intramedullary lesions confined to the bone. It may demonstrate soft tissue extension; therefore, it may be useful in guiding percutaneous biopsy.
Ultrasonography is useful as a means of assessing the thickness of the cartilage cap overlying an osteochondroma, although access to the lesion may be difficult in certain areas. If the cap measures more than 1.5 cm in a skeletally mature patient, transformation to chondrosarcoma may have occurred.
Nuclear Imaging
Findings
Central chondrosarcomas typically show significantly increased uptake of the radioisotope on isotopic bone scans, but differentiation between chondrosarcoma and enchondroma is unreliable. Uptake on isotopic bone scanning may indicate either metabolic activity in an osteochondroma or malignant transformation; these two conditions cannot be distinguished on the basis of such a finding. In the absence of an increase in uptake, malignancy is highly unlikely.
Degree of Confidence
See Findings, above.
Angiography
Findings
Angiography is generally not required in the staging of chondrosarcoma.
Intervention
Histologic confirmation of the nature of a lesion is required for optimal management. Although biopsy is often performed as an open surgical procedure, percutaneous biopsy with imaging guidance may be helpful in certain scenarios. Ultrasonography is useful for guiding needle biopsy of the soft tissue component of a tumor. CT-guided percutaneous biopsy of bone lesions may be helpful, but the pathologic differentiation between benign and low-grade malignant chondrosarcomas is difficult. Needle biopsy samples may be inadequate for making a precise pathologic diagnosis, and open surgical biopsy is often performed.
The mainstay of treatment is surgical resection because chondrosarcomas respond poorly to chemotherapy or radiation therapy.
Medicolegal Pitfalls
- Although chondrosarcomas are malignant tumors, they often have features suggestive of a benign lesion.
- Clinicians should be cautious not to mistake chondrosarcomas for benign lesions because of their well-circumscribed appearance and the lack of periosteal reaction.
Multimedia
Media file 1: Frontal radiograph of the left fibula head demonstrates a lucent lesion that contains the typical chondroid matrix calcification. Low-grade tumor.
Media file 2: Frontal radiograph of the left acetabulum demonstrates an expansile lucent lesion with no internal matrix calcification. Low-grade central tumor.
Media file 3: Bone-window CT scan of left acetabulum demonstrates matrix calcification in the expansile lucent lesion in the anterior column (see also Images 2, 4, and 5 in Multimedia Section). Low-grade central tumor.
Media file 4: T2-weighted axial MRI of the pelvis demonstrates the high signal intensity of the acetabular lesion (same patient as in Images 2-3, 5 in Multimedia). Low-grade central tumor.
Media file 5: T1-weighted axial MRI of the pelvis demonstrates the low signal intensity of the acetabular lesion (same patient as in Images 2-4 in Multimedia). Low-grade central tumor.
Media file 6: Frontal radiograph of right side of upper abdomen demonstrates a destructive, expansile lesion of the 12th rib. The lesion contains irregular calcification. High-grade central tumor.
Media file 7: CT of the right side of the upper abdomen demonstrates the expansile tumor with a large associated soft tissue mass containing foci of calcification (see also Image 6 in Multimedia Section). High-grade central tumor.
Media file 8: Frontal radiograph of the pelvis demonstrates extensive calcification overlying the left ilium and in the lateral soft tissues. No bone destruction is shown. High-grade secondary peripheral tumor.
Media file 9: CT scan of the pelvis demonstrates a large soft tissue mass that contains calcification arising from a broad-based sessile osteochondroma on the posterior aspect of the ilium (see also Images 8 and 10 in Multimedia Section). High-grade secondary peripheral tumor.
Media file 10: T2-weighted axial MRI of the pelvis demonstrates a lobulated high-signal-intensity soft tissue with local-signal-intensity septa arising from the osteochondroma on the posterior aspect of the ilium (same patient as in Image 9 in Multimedia). Several areas of low signal intensity are shown; these correspond to focal areas of dense calcification. This appearance is typical of cartilaginous material. High-grade secondary peripheral tumor.
Media file 11: Lateral radiograph of the distal femur in a patient with hereditary multiple exostoses. Several osteochondromas of varying appearances arise from the metaphyseal region; these typically grow away from the joint. Soft tissue calcification is shown overlying the most posterior osteochondroma. High-grade secondary peripheral tumor.
Media file 12: CT scan of the distal femur demonstrates a broad-based osteochondroma with a thick overlying soft tissue cap that contains focal calcification. The metaphyseal contour is irregular because of the presence of several other osteochondromas in this patient with hereditary multiple exostoses (see also Images 11 and 13 in Multimedia Section). High-grade secondary peripheral tumor.
Media file 13: Fast spin-echo T2-weighted axial MRI of the distal femur in a patient with hereditary multiple exostoses (same patient as in Images 11-12 in Multimedia). Image demonstrates the thick cartilage cap overlying a broad-based osteochondroma. Areas of focal reduced signal intensity in the cartilage cap correspond to foci of dense calcification. High-grade secondary peripheral tumor.
References
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Giuffrida AY, Burgueno JE, Koniaris LG, Gutierrez JC, Duncan R, Scully SP. Chondrosarcoma in the United States (1973 to 2003): an analysis of 2890 cases from the SEER database. J Bone Joint Surg Am. May 2009;91(5):1063-72. [Medline].
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Langheinrich AC, Stolle C, Kampschulte M, Lommel D, Rau WS, Bassaly B. Diagnostic Value of Ex-Vivo Three-Dimensional Micro-Computed Tomography Imaging of Primary Nonhematopoietic Human Bone Tumors: Osteosarcoma versus Chondrosarcoma. Acta Radiol. Jul 11 2008;1-8. [Medline].
Murata H, Horie N, Matsui T, Akai T, Ueda H, Oshima Y, et al. Clinical usefulness of thallium-201 scintigraphy and magnetic resonance imaging in the diagnosis of chondromyxoid fibroma. Ann Nucl Med. Apr 2008;22(3):221-4. [Medline].
Bauer HC, Brosjo O, Kreicbergs A, Lindholm J. Low risk of recurrence of enchondroma and low-grade chondrosarcoma in extremities. 80 patients followed for 2-25 years. Acta Orthop Scand. Jun 1995;66(3):283-8. [Medline].
Walden MJ, Murphey MD, Vidal JA. Incidental enchondromas of the knee. AJR Am J Roentgenol. Jun 2008;190(6):1611-5. [Medline].
Keywords
chondrosarcoma, bone tumors, primary bone tumor, bone malignancy, primary osseous neoplasms, sarcomas, chondroid, conventional chondrosarcomas, central chondrosarcoma, peripheral chondrosarcoma, enchondroma, osteochondroma, clear cell chondrosarcoma, myxoid chondrosarcoma, mesenchymal chondrosarcoma, dedifferentiated chondrosarcoma
Contributor Information and Disclosures
Author
Geoff Hide, MBBS, MRCP, FRCR, Consultant Musculoskeletal Radiologist, Department of Radiology, Freeman Hospital; Honorary Clinical Lecturer, Faculty of Medical Sciences, University of Newcastle upon Tyne
Geoff Hide, MBBS, MRCP, FRCR is a member of the following medical societies: British Medical Association, Royal College of Physicians, and Royal College of Radiologists
Disclosure: Nothing to disclose.
Medical Editor
Michael A Bruno, MD, Associate Professor, Departments of Radiology and Medicine, Pennsylvania State University College of Medicine; Director, Radiology Quality Management Services, Milton S Hershey Medical Center, Pennsylvania State University College of Medicine
Michael A Bruno, MD is a member of the following medical societies: American College of Radiology, American Roentgen Ray Society, Association of University Radiologists, Radiological Society of North America, Society of Nuclear Medicine, and Society of Skeletal Radiology
Disclosure: Nothing to disclose.
Pharmacy Editor
Bernard D Coombs, MB, ChB, PhD, Consulting Staff, Department of Specialist Rehabilitation Services, Hutt Valley District Health Board, New Zealand
Disclosure: Nothing to disclose.
Managing Editor
Murali Sundaram, MBBS, FRCR, FACR, Consulting Staff, Department of Diagnostic Radiology, The Cleveland Clinic Foundation
Disclosure: Nothing to disclose.
CME Editor
Robert M Krasny, MD, Resolution Imaging Medical Corporation
Robert M Krasny, MD is a member of the following medical societies: American Roentgen Ray Society and Radiological Society of North America
Disclosure: Nothing to disclose.
Chief Editor
Felix S Chew, MD, MBA, EdM, Professor, Department of Radiology, Vice Chairman for Radiology Informatics, Section Head of Musculoskeletal Radiology, University of Washington
Felix S Chew, MD, MBA, EdM is a member of the following medical societies: American Roentgen Ray Society, Association of University Radiologists, and Radiological Society of North America
Disclosure: Nothing to disclose.