Introduction
Background
Chondromyxoid fibroma (CMF) is a rare benign tumor of the bone that was described by Jaffe and Lichenstein in 1948.1 CMF is most often found in the long tubular bones, especially the tibia and femur near the knee joint. CMF occurs predominantly in younger patients in the second or third decade of life.2,3
Pathophysiology
The etiology chondromyxoid fibroma (CMF) is unknown; however, one report has pointed to an error in chromosome 6.4 The tumor arises from the cartilage-forming connective tissue of the marrow space. Histologically, as its name implies, this benign cartilaginous neoplasm consists of chondroid, myxoid, and fibrous tissue in variable amounts, and microscopic evaluation of a wide area of the tumor may be necessary to identify all of the tissue subtypes.
Radiograph of the proximal tibia of a 16-year-old boy reveals a large, lucent, slightly expansile, eccentric, metaphyseal lesion with thin sclerotic borders. Pathologic analysis helped confirm a diagnosis of chondromyxoid fibroma.
Anteroposterior radiograph of the distal femur of a 14-year-old girl reveals a large, expansile, bubbly, eccentric, metadiaphyseal lesion. Pathologic analysis helped confirm a diagnosis of chondromyxoid fibroma.
Osteoclast-like giant cells may also be present, as may small cysts and hemorrhagic zones. Focal calcification is found microscopically in approximately one fourth of patients, although any gross evidence of calcification is rare.
Frequency
United States
Chondromyxoid fibromas are among the rarest of the bone tumors, representing less than 1% of primary osseous neoplasms (approximately 2% of benign bone tumors).
International
No data suggest that the international frequency of chondromyxoid fibroma is different from the frequency of incidence in the United States.
Mortality/Morbidity
Chondromyxoid fibroma (CMF) may present with a pathologic fracture through the tumor, which may lead to morbidity. If managed appropriately, the lesion is not fatal. If left undiagnosed, the tumor continues to grow, occasionally infiltrating the surrounding soft tissues and causing further damage. Although CMF is considered to be a benign lesion, rare instances of malignancy have been reported. Malignant degeneration following radiation therapy in patients with CMF also has been reported; therefore, irradiation is contraindicated as a mode of therapy.
Race
No racial predilection has been observed.
Sex
Several reports claim a predilection in males, with a male-to-female ratio of 1.5-2:1. Other authors deny a sex predilection.
Age
The tumor is found predominantly in patients in the second and third decades of life; more than 80% of cases occur in patients younger than 36 years (although patients as young as 3 years and as old as 79 years have been reported). A second incidence peak may occur in patients aged 50-70 years.
Anatomy
Most chondromyxoid fibromas (75%) occur in the bones of the lower extremity, particularly around the knee joint. CMF is localized to the femur (see Image 3) and tibia (see Image 13) in 50% of patients. The most common site is the proximal tibia, which accounts for approximately 30% of cases. The humerus, radius, and ulna also are affected, although reported percentages vary widely from study to study because of the rarity of the lesion. In addition, the small bones of the foot are relatively common sites, and lesions of the hands, skull, spine, and pelvis (see Image 25) have been reported.5
Lateral radiograph of the distal femur of a 14-year-old girl (same patient as in Image 2 in Multimedia). A large expansile chondromyxoid fibroma is seen.
Anteroposterior radiograph of the proximal tibia of a 36-year-old man reveals a well-defined lucent lesion in the metadiaphysis with sclerotic margins. Pathologic analysis helped confirm a diagnosis of chondromyxoid fibroma.
T1-weighted coronal MR image of the sacroiliac joints in a 20-year-old woman reveals a chondromyxoid fibroma with low signal intensity in the right ilium (same patient as in Images 23-24 in Multimedia).
Within the bone, the tumor typically originates in the metaphysis close to the physis. The tumor may extend into the epiphysis, the diaphysis, or both. Apophyses also may be affected (eg, the greater trochanter of the femur). In the long bones, the tumor is usually eccentric and ovoid in shape (see Image 14), with the long axis paralleling the length of the bone. In smaller bones, the tumor may occupy the entire volume of bone.
Presentation
Pain and local soft-tissue swelling are the most common presenting complaints (approximately 85% and 65% of patients, respectively). However, the duration of pain and swelling is quite variable; duration of pain averages approximately 22 months and duration of swelling averages approximately 10 months. This relatively long duration of symptoms denotes a slow tumor growth rate. Pathologic fracture is observed in some patients with painful tumors. Asymptomatic tumors may occasionally be detected incidentally on radiographs.6
Preferred Examination
Conventional radiography provides the most useful diagnostic information of any imaging modality; however, definitive diagnosis can only be made using analysis of biopsy specimens. Unless contraindicated, magnetic resonance imaging (MRI) is recommended over computed tomography (CT) for delineation of tumor extent before surgery (see Image 9).
Limitations of Techniques
Although findings on conventional radiographs may suggest the diagnosis of CMF, definitive diagnosis requires an analysis of biopsy specimens.
Differential Diagnoses
Other Problems to Be Considered
Desmoplastic fibroma
More on Chondromyxoid Fibroma |
 Overview: Chondromyxoid Fibroma |
| Imaging: Chondromyxoid Fibroma |
| Follow-up: Chondromyxoid Fibroma |
| Multimedia: Chondromyxoid Fibroma |
| References |
| Further Reading |
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References
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Further Reading
Related eMedicine topics
Chondromyxoid Fibroma (from Orthopedic Surgery)
Fibroma
Aneurysmal Bone Cyst
Osteoblastoma
Histology of Bone
Keywords
chondromyxoid fibroma, CMF, fibromyxoid chondroma, myxofibrous chondroma, primary osseous neoplasm, benign bone tumor, osseous tumor, lower extremity tumor
