Introduction
Frontal radiograph of the left fibula head demonstrates a lucent lesion that contains the typical chondroid matrix calcification. Low-grade tumor.
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
Related eMedicine topics:
Chondrosarcoma (from Orthopedic Surgery)
Chondroma
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.4,5,6,7
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
More on Chondrosarcoma |
 Overview: Chondrosarcoma |
| Imaging: Chondrosarcoma |
| Follow-up: Chondrosarcoma |
| Multimedia: Chondrosarcoma |
| References |
| Further Reading |
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References
Flemming DJ, Murphey MD. Enchondroma and chondrosarcoma. Semin Musculoskelet Radiol. 2000;4(1):59-71. [Medline].
Marco RA, Gitelis S, Brebach GT, Healey JH. Cartilage tumors: evaluation and treatment. J Am Acad Orthop Surg. Sep-Oct 2000;8(5):292-304. [Medline].
Gelderblom H, Hogendoorn PC, Dijkstra SD, van Rijswijk CS, Krol AD, Taminiau AH, et al. The clinical approach towards chondrosarcoma. Oncologist. Mar 2008;13(3):320-9. [Medline].
Murphey MD, Flemming DJ, Boyea SR, et al. Enchondroma versus chondrosarcoma in the appendicular skeleton: differentiating features. Radiographics. Sep-Oct 1998;18(5):1213-37; quiz 1244-5. [Medline].
Resnik D, Kyriakos M, Greenaway GD. Tumors and tumor-like lesions of bone: imaging and pathology of specific lesions. In: Diagnosis of Bone and Joint Disorders. 4th ed. Philadelphia, Pa: WB Saunders Co;2002: 3897-919.
Wang XL, De Beuckeleer LH, De Schepper AM, Van Marck E. Low-grade chondrosarcoma vs enchondroma: challenges in diagnosis and management. Eur Radiol. 2001;11(6):1054-7. [Medline].
Saki N, Akhlagh SN, Mostofi NE, Ahmadi K. Chondrosarcoma of the hyoid bone: imaging, surgical, and histopathologic correlation. Laryngoscope. Jul 2008;118(7):1211-3. [Medline].
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].
Further Reading
Related eMedicine topics:
Chondrosarcoma (from Orthopedic Surgery)
Chondroma
Related Medscape topics:
Radiology CME and News
Specialty Site Radiology
Specialty Site Oncology
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
