Chondromyxoid fibroma is basically a benign tumour, but it has the likelihood of being aggressive. It is derived from cartilage, accounting for approximately 1% of all bone tumours. The Chondromyxoid fibroma typically is known to affect the metaphysis of long bones of younger patients. Another increase of incidence of Chondromyxoid Fibroma is seen in the 6th-7th decade of life.
How is chondromyxoid fibroma different from chondroblastoma ?
Chondromyxoid fibroma was the term coined by Jaffe and Lichtenstein (1948) to describe a group of eight bone tumours which had at first been diagnosed as chondrosarcoma, but later they recognised these tumours had a distinct histological pattern.
Despite the malignant appearance of several of the cells they gauged the tumours to be benign and hence worthy of a new classification.
Chondromyxoid Fibroma is benign whereas Chondrosarcoma is malignant.
What is the clinical presentation of chondromyxoid fibroma (CMF) ?
Clinically, the patients’ complaints are not so specific. Usually there is pain and a slowly increasing local swelling. It can be tender on pressure. Sometimes it may affect the movement of the person, causing limping, but there is usually no significant involvement of a joint.
A related pathologic fracture can occur in about 5% of cases.
The commonest site of occurrence of the tumour is the metaphysis, adjoining the epiphyseal growth plate. This collaborates with the theory that the tumour is derived from cartilaginous remnants at these areas. Epiphyseal involvement is very uncommon. Most occur in the lower limb, particularly around the knee but also in the bones of the foot. The proximal end of the tibia is by far the most common site.
How is chondromyxoid fibroma diagnosed ?
It is best diagnosed by magnetic resonance imaging (MRI). A MRI Scan is useful in characterizing the tumour and in documenting the extent of soft tissue involvement.
Radiographically, the appearances in many cases are characteristic. There is a well defined bone defect in an eccentric position in or near the metaphysis of a long bone. A typical radiolucent lesion (this indicates bone destruction) is seen with well-defined sclerotic margins and the presence of a thinned cortex.
Recognition of the tumour is governed mostly by its histological appearance.
Histopathologically, Chondromyxoid Fibroma has a cartilage-like matrix composed of three elements in varying proportions- the cartilaginous, the fibrous, and last but not least, the myxoid areas.
There are occasional foci of calcification, giant cells and cells with irregular nuclei.
What can chondromyxoid fibroma be confused with ?
It is generally decided that the tumour is definitely benign. But the tumour cells can look deceptively malignant and may guide the pathologist to a mistaken diagnosis of chondrosarcoma.
Differentiating the Chondromyxoid Fibroma from an aneurysmal bone cyst (ABC) may be difficult, as well.
How can chondromyxoid fibroma be treated ?
Preferred treatment is complete local excision with tumour-free margins. Intralesional curettage shows a local recurrence rate of roughly 25%. Even radiation therapy may be useful in nonresectable cases but bear the risk of inducing other malignancies.
Medical treatment is usually not needed for chondromyxoid fibromas (CMFs) unless painkillers have to be taken for the pain.
What is the risk of recurrence for chondromyxoid fibroma ?
Despite the benign nature of the tumour, its local aggressiveness should not be underestimated. Age seems to have a significant influence on the recurrence rate.
Planning the extent of resection with MRI may result in a reduction of the recurrence rate.
Although it is a benign tumour, recurrence after intralesional treatment may range from 10% to 80%. To conclude, the recurrence rate is lesser with resection using wide margins, even though this cannot be done in all of the anatomical regions.
What is the risk of malignant transformation for chondromyxoid fibroma ?
Malignant transformation appears to be very exceptional, with only isolated cases having been reported in literature.
What is the care to be taken after the removal of the lesion ?
Regular check-ups need to be undergone to rule out the risk of recurrence or very rarely, malignant transformation.
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Disclaimer: The content provided here is meant for general informational purposes only and hence SHOULD NOT be relied upon as a substitute for sound professional medical advice, care or evaluation by a qualified doctor/physician or other relevantly qualified healthcare provider.
