AUTHOR AND EDITOR INFORMATION

Section 1 of 12  

• Authors and Editors
• Introduction
• Differentials
• Radiograph
• CT SCAN
• Mri
• Ultrasound
• Nuclear Medicine
• Angiography
• Intervention
• Multimedia
• References

INTRODUCTION

Section 2 of 12  

• Authors and Editors
• Introduction
• Differentials
• Radiograph
• CT SCAN
• Mri
• Ultrasound
• Nuclear Medicine
• Angiography
• Intervention
• Multimedia
• References

Background

Chondromyxoid fibroma (CMF) is a rare benign tumor of the bone that was described by Jaffe and Lichenstein in 1948. 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.

Pathophysiology

The etiology CMF is unknown; however, a recent report points to an error in chromosome 6 (Safar et al, 2000). 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. 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

CMFs 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 CMF is different from the frequency of incidence in the United States.

Mortality/Morbidity

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 CMFs (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.

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.

Clinical Details

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.

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.

DIFFERENTIALS

Section 3 of 12  

• Authors and Editors
• Introduction
• Differentials
• Radiograph
• CT SCAN
• Mri
• Ultrasound
• Nuclear Medicine
• Angiography
• Intervention
• Multimedia
• References

Other Problems to be Considered

Desmoplastic fibroma

RADIOGRAPH

Section 4 of 12  

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• Introduction
• Differentials
• Radiograph
• CT SCAN
• Mri
• Ultrasound
• Nuclear Medicine
• Angiography
• Intervention
• Multimedia
• References

Findings

On conventional radiographs, a CMF usually appears as a well-marginated, expansile, eccentric, lucent medullary lesion in the metaphysis of a long bone, ranging in length from 3-10 cm (see Image 1). The tumor may extend into the diaphysis (see Image 2) or, uncommonly, into the epiphysis (see Image 6). CMF may rarely be purely diaphyseal (see Image 10), but it is never solely epiphyseal. The tumor may replace the bulk of a smaller bone. Smaller CMFs may appear to arise from the cortex of bone, and juxtacortical (exophytic) tumors have been reported.

Smaller tumors are usually round with a thin sclerotic margin (see Image 5) and uncommonly contain visible calcification or trabeculation. In larger lesions, remnants of cortical bone reinforcing the tumor at the periphery can appear on radiographs as trabeculation (see Image 23). These osseous ridges along the periphery are also responsible for the bubbly cystic radiographic appearance of CMF (see Image 22). A sclerotic scalloped border is typical. Compared with other cartilaginous tumors, the matrix of CMF uncommonly appears calcified on conventional radiographs (see Image 18).

Degree of Confidence

An eccentric, medullary, bubbly-appearing, metaphyseal lesion with scalloped sclerotic borders should prompt the radiologist to consider CMF in the differential diagnosis, particularly if the lesion is found in the proximal tibia; however, diagnosing CMF with a high degree of confidence by using imaging studies may be difficult because of the rarity of the tumor. An analysis of a biopsy specimen is always necessary for a definitive diagnosis.

CT SCAN

Section 5 of 12  

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• Introduction
• Differentials
• Radiograph
• CT SCAN
• Mri
• Ultrasound
• Nuclear Medicine
• Angiography
• Intervention
• Multimedia
• References

Findings

After conventional radiography, CT scans may be used to further study the nature and extent of the suspected CMF (see Images 11-12). CT is the best imaging modality for detecting sclerotic margins and ridges (see Image 24) and matrix mineralization (see Image 19), and CT findings can depict the cortical integrity of the lesion. CT scans may show calcification within the tumor that is not visible on conventional radiographs.

Degree of Confidence

CT findings may reveal calcifications within the lesion that are not apparent on conventional radiographs; therefore, CT findings may increase the suspicion that a lesion is cartilaginous. Otherwise, CT scans add little to the diagnosis.

MRI

Section 6 of 12  

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• Introduction
• Differentials
• Radiograph
• CT SCAN
• Mri
• Ultrasound
• Nuclear Medicine
• Angiography
• Intervention
• Multimedia
• References

Findings

MRI may be used in the management of CMF to observe the true extent of the lesion, so that complete resection may be planned and potential recurrence may be avoided.

MRI findings of CMF are nonspecific. The tumor typically demonstrates low signal intensity on T1-weighted images (see Image 7, Image 16) and heterogeneous high signal intensity on T2-weighted images (see Image 26). Smaller lesions, as well as some larger lesions, may demonstrate a homogeneously bright signal on T2-weighted images, often with a hypointense rim (see Image 17). Enhancement following intravenous administration of gadolinium is typically heterogeneous (see Image 8), often most prominent along the vascular borders of the tumor. Heterogeneity is believed to be the result of varying amounts of chondroid, myxoid, and fibrous tissues in the tumor, as well as any underlying cystic and/or hemorrhagic components.

Gadolinium-based contrast agents (gadopentetate dimeglumine [Magnevist], gadobenate dimeglumine [MultiHance], gadodiamide [Omniscan], gadoversetamide [OptiMARK], gadoteridol [ProHance]) have recently been linked to the development of nephrogenic systemic fibrosis (NSF) or nephrogenic fibrosing dermopathy (NFD). For more information, see the eMedicine topic Nephrogenic Fibrosing Dermopathy. The disease has occurred in patients with moderate to end-stage renal disease after being given a gadolinium-based contrast agent to enhance MRI or MRA scans.

As of late December 2006, the FDA had received reports of 90 such cases. Worldwide, over 200 cases have been reported, according to the FDA. NSF/NFD is a debilitating and sometimes fatal disease. Characteristics include red or dark patches on the skin; burning, itching, swelling, hardening, and tightening of the skin; yellow spots on the whites of the eyes; joint stiffness with trouble moving or straightening the arms, hands, legs, or feet; pain deep in the hip bones or ribs; and muscle weakness. For more information, see the FDA Public Health Advisory or Medscape.

Degree of Confidence

MRI findings of CMF are nonspecific and typically do not alter the degree of confidence in the diagnosis. The primary role of MRI is in preoperative planning (ie, evaluation of the extent of the tumor).

ULTRASOUND

Section 7 of 12  

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• Introduction
• Differentials
• Radiograph
• CT SCAN
• Mri
• Ultrasound
• Nuclear Medicine
• Angiography
• Intervention
• Multimedia
• References

Findings

Ultrasound currently has no role in the diagnosis of CMF.

NUCLEAR MEDICINE

Section 8 of 12  

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• CT SCAN
• Mri
• Ultrasound
• Nuclear Medicine
• Angiography
• Intervention
• Multimedia
• References

Findings

Although increased radionuclide activity is demonstrated in CMFs on bone scans (see Image 21), nuclear medicine procedures are of limited use in the diagnosis or management of these lesions. The typically eccentric location of a CMF may be evident if the lesion is relatively small (see Image 15). Smaller lesions may be subtle if located adjacent to a growing physis or joint that typically accumulates radiotracer (eg, sacroiliac joint) (see Image 27). Increased flow may be apparent on the angiographic portion of a 3-phase study (see Image 20). Bone scintigraphy may be used to exclude the possibility of multiple lesions, which are highly uncharacteristic of CMF (see Image 4).

Degree of Confidence

Nuclear medicine studies add little to the degree of confidence in the diagnosis, although multiple lesions on a bone scan is highly uncharacteristic of a CMF.

ANGIOGRAPHY

Section 9 of 12  

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• Differentials
• Radiograph
• CT SCAN
• Mri
• Ultrasound
• Nuclear Medicine
• Angiography
• Intervention
• Multimedia
• References

Findings

Angiography is of limited use in the diagnosis of CMF. Angiographic appearances are nonspecific, with the tumor demonstrating either minimal neovascularity or no internal vascularity, with or without surrounding vascular tissue. Angiography may be used to define surrounding vasculature or for planning embolization (uncommon).

Degree of Confidence

Angiography typically does not alter the degree of confidence in the diagnosis, but it may be used as a preoperative study.

INTERVENTION

Section 10 of 12  

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• Multimedia
• References

Treatment in patients with CMF usually involves curettage or en bloc resection, with a preference for en bloc resection. Radiation therapy is contraindicated because of the risk of inducing malignancy. Periodic follow-up studies are indicated because of the significant recurrence rate of CMFs.

Medical/Legal Pitfalls

• Patients with CMF present with generic symptoms that are very similar to those of other benign and malignant bone tumors.
• Imaging findings may be suggestive of CMF, but ultimately, an analysis of biopsy specimens is required for a definitive diagnosis.
• The lesion shares some imaging features with chondrosarcoma, which is treated more aggressively.

Special Concerns

• The recurrence of CMF after curettage has been documented in numerous patients. Most recurrent tumors are observed in patients younger than 20 years. Recurrent tumors retain the histologic and gross characteristics of the original tumor.

MULTIMEDIA

Section 11 of 12  

• Authors and Editors
• Introduction
• Differentials
• Radiograph
• CT SCAN
• Mri
• Ultrasound
• Nuclear Medicine
• Angiography
• Intervention
• Multimedia
• References

Media file 1:  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.
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Media file 2:  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.
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Media type:  X-RAY

Media file 3:  Lateral radiograph of the distal femur of a 14-year-old girl (same patient as in Image 2). A large expansile chondromyxoid fibroma is seen.
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Media type:  X-RAY

Media file 4:  Delayed bone scan image of a 14-year-old girl (same patient as in Images 2-3). Activity in the distal left femur is increased at the site of a chondromyxoid fibroma. No additional sites of abnormal uptake are seen.
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Media type:  Image

Media file 5:  Chondromyxoid fibroma. Radiograph of the proximal tibia of a 37-year-old man shows a small, lucent, eccentric, metaphyseal lesion with a thin sclerotic margin. No intervention or additional imaging was performed at the time.
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Media file 6:  Radiograph of the proximal tibia obtained 5 years after the radiograph in Image 5 was obtained. The lucent metaphyseal lesion has grown and currently extends into the proximal tibial epiphysis. Pathologic analysis helped confirm a diagnosis of chondromyxoid fibroma.
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Media type:  X-RAY

Media file 7:  T1-weighted transverse MR image of the proximal tibia of a 37-year-old man (same patient as in Images 5-6). The eccentric lesion with low signal intensity situated in the anterior tibia corresponds to the location of the chondromyxoid fibroma in this patient.
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Media type:  MRI

Media file 8:  Fat-saturated T1-weighted transverse MR image of the proximal tibia of a 37-year-old man obtained after the intravenous administration of gadolinium (same patient as in Images 5-7). The chondromyxoid fibroma is heterogeneously enhancing.
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Media type:  MRI

Media file 9:  Fat-suppressed T2-weighted coronal MR image of the knee of a 37-year-old man (same patient as in Images 5-8). High signal intensity in the medial aspect of the proximal tibia corresponds to the location of the chondromyxoid fibroma in this patient.
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Media type:  MRI

Media file 10:  Radiograph of the tibia of a 15-year-old girl reveals a lucent, slightly expansile, diaphyseal lesion. Pathologic analysis helped confirm a diagnosis of chondromyxoid fibroma.
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Media type:  X-RAY

Media file 11:  Scout image from a CT examination of the legs of a 15-year-old girl reveals a lucent diaphyseal lesion with sclerotic margins; this lesion represents a chondromyxoid fibroma in this patient (same patient as in Image 10).
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Media type:  CT

Media file 12:  CT scan of the legs of a 15-year-old girl reveals replacement of the normal fatty marrow by a chondromyxoid fibroma (same patient as in Images 10-11). Trabeculation is evident.
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Media type:  CT

Media file 13:  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.
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Media type:  X-RAY

Media file 14:  Lateral radiograph of the tibia of a 36-year-old man (same patient as in Image 13). A chondromyxoid fibroma is situated anteriorly within the tibia along the cortex.
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Media file 15:  Delayed bone scan image of a 36-year-old man reveals increased activity in the anterior aspect of the proximal tibia, corresponding to a chondromyxoid fibroma in this patient (same patient as in Images 13-14).
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Media type:  Image

Media file 16:  T1-weighted transverse MR image of the proximal tibia of a 36-year-old man reveals a chondromyxoid fibroma with low signal intensity (same patient as in Images 13-15).
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Media type:  MRI

Media file 17:  T2-weighted transverse MR image of the tibia of a 36-year-old man reveals a chondromyxoid fibroma with high signal intensity (same patient as in Images 13-16).
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Media type:  MRI

Media file 18:  Radiograph of the distal tibia of a 16-year-old boy reveals a lucent eccentric metaphyseal lesion with a thin sclerotic margin. Pathologic analysis helped confirm a diagnosis of chondromyxoid fibroma.
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Media type:  X-RAY

Media file 19:  CT scan of the distal tibia of a 16-year-old boy reveals calcific matrix in a chondromyxoid fibroma (same patient as in Image 18).
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Media type:  CT

Media file 20:  Angiographic phase images from a bone scan of a 16-year-old boy reveal increased flow to the site of the chondromyxoid fibroma in the distal right tibia (same patient as in Images 18-19).
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Media type:  Image

Media file 21:  Delayed bone scan image reveals increased activity in the distal right tibia, corresponding to a chondromyxoid fibroma (same patient as in Images 18-20).
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Media type:  Image

Media file 22:  Radiograph of the first metatarsal of a 16-year-old boy reveals a bubbly, expansile, lytic lesion in the distal metadiaphysis. Pathologic analysis helped confirm a diagnosis of chondromyxoid fibroma.
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Media file 23:  Radiograph of the right sacroiliac joint of a 20-year-old woman reveals a bubbly lucent lesion of the medial right ilium with sclerotic margins. Pathologic analysis helped confirm a diagnosis of chondromyxoid fibroma.
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Media file 24:  CT scan of the pelvis of a 20-year-old woman reveals a right iliac chondromyxoid fibroma (same patient as in Image 23). Trabeculation is evident.
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Media type:  CT

Media file 25:  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).
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Media type:  MRI

Media file 26:  T2-weighted transverse MR image of the pelvis of a 20-year-old woman (same patient as in Images 23-25). A right iliac chondromyxoid fibroma demonstrates heterogeneous high signal intensity.
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Media type:  MRI

Media file 27:  Delayed bone scan image of the pelvis (posterior view) of a 20-year-old woman (same patient as in Images 23-26). Activity at the right sacroiliac joint is slightly increased, corresponding to a chondromyxoid fibroma in this patient.
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Media type:  Image

REFERENCES

Section 12 of 12

• Authors and Editors
• Introduction
• Differentials
• Radiograph
• CT SCAN
• Mri
• Ultrasound
• Nuclear Medicine
• Angiography
• Intervention
• Multimedia
• References

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• Murphy NB, Price CH. The radiological aspects of chondromyxoid fibroma of bone. Clin Radiol. Apr 1971;22(2):261-9. [Medline].
• Park JM, Woo YK, Kang MI, et al. Oncogenic osteomalacia associated with soft tissue chondromyxoid fibroma. Eur J Radiol. Aug 2001;39(2):69-72. [Medline].
• Park SH, Kong KY, Chung HW, et al. Juxtacortical chondromyxoid fibroma arising in an apophysis. Skeletal Radiol. Aug 2000;29(8):466-9. [Medline].
• RCNA. Chondromyxoid fibroma. Radiol Clin North Am. 1993;31:251-3.
• Rahimi A, Beabout JW, Ivins JC, Dahlin DC. Chondromyxoid fibroma: a clinicopathologic study of 76 cases. Cancer. Sep 1972;30(3):726-36. [Medline].
• Safar A, Nelson M, Neff JR. Recurrent anomalies of 6q25 in chondromyxoid fibroma. Hum Pathol. Mar 2000;31(3):306-11.
• Tarhan NC, Yologlu Z, Tutar NU, et al. Chondromyxoid fibroma of the temporal bone: CT and MRI findings. Eur Radiol. 2000;10(10):1678-80. [Medline].
• Yamaguchi T, Dorfman HD. Radiographic and histologic patterns of calcification in chondromyxoid fibroma. Skeletal Radiol. Oct 1998;27(10):559-64. [Medline].

Article Last Updated: Feb 21, 2007