Chondromyxoid fibromas (CMFs) are extremely rare, benign cartilaginous neoplasms that account for <1% of all bone tumours.
The majority of cases occur in the second and third decades, with ~75% of cases occurring before the age of 30 years 1,12-15. There is no recognised gender predilection 18. However, examples have been seen in patients up to the age of 75 years. In some series, there is a male predilection, whilst in others, no such distribution is found 2,12.
Typically patients present with progressive pain, often long-standing and/or bony swelling and restricted range of movement in affected limb 3,12. The latter is most often the case in bones with little overlying soft tissues (e.g. short tubular bones of the hands and feet).
The tumour comprises of a variable combination of chondroid, myxoid, and fibrous tissue components organised in a pseudolobulated architecture 20.
On gross examination, they are typically seen as solid glistening tan-gray intraosseous masses.
Occasional osteoclast-like giant multinucleated cells are encountered particularly at the periphery. Most cells are morphologically bland, and mitotic figures are rare or absent 13.
Most chondromyxoid fibromas are located in the metaphyseal region of long bones (60%), and may extend to the epiphyseal line and even rarely abut the articular surface 3,12. They are almost never just epiphyseal 3.
The classical site is the upper one-third of the tibia, which accounts for 25% of all cases, with the small tubular bones of the foot, the distal femur and pelvis being other relatively common locations 12.
- often seen as a lobulated, eccentric radiolucent lesion
- long axis parallel to long axis of long bone
- no periosteal reaction (unless a complicating fracture present)
- geographic bone destruction: almost 100%
- well defined sclerotic margin: ~85%
- presence of septations (pseudotrabeculation): ~60% 2
- presence of matrix calcification in small proportion cases: 12.5%1
MR features are often not particularly specific. Signal characteristics include:
- T1: low signal
T1 C+ (Gd)
- the majority (~70%) tend to show peripheral nodular enhancement
- ~30% diffuse contrast enhancement and this can be either homogeneous or heterogeneous 19
- T2: high signal
Treatment and prognosis
They are benign lesions and malignant degeneration is rare. They are usually treated with curettage which however have a high recurrence rate of 25%. As such if an en-bloc resection is possible this is advisable 14.
History and etymology
It is thought to have been initially described by H L Jaffe and L Lichtenstein in 1948 7.
General imaging differential considerations include
- aneurysmal bone cyst (ABC)
- giant cell tumour of bone (GCT)
- non-ossifying fibroma: younger age group
- chondroblastoma: younger age group
The differential diagnosis for bone tumours is dependent on the age of the patient, with a very different set of differentials for the paediatric patient.
- bone-forming tumours
- chondromyxoid fibroma
- fibrous bone lesions
- bone marrow tumours
- other bone tumours or tumour-like lesions
- aneurysmal bone cyst
- benign fibrous histiocytoma
- giant cell tumour of bone
- Gorham massive osteolysis
- haemophilic pseudotumour
- intradiploic epidermoid cyst
- intraosseous lipoma
- cockade sign
- musculoskeletal angiosarcoma
- primary intraosseous haemangioma
- simple bone cyst
- impending fracture risk
- 1. Grainger RG, Allison D, Adam A et-al. Grainger's and Allison's Diagnostic Radiology, A Textbook of Medical Imaging. (2008) ISBN:0443101639. Read it at Google Books - Find it at Amazon
- 2. Dähnert W. Radiology Review Manual. Lippincott Williams & Wilkins. (2011) ISBN:1609139437. Read it at Google Books - Find it at Amazon
- 3. Aids to Radiological Differential Diagnosis. edited by Stephen G. Davies MA MB BChir MRCP FRCR Saunders Ltd. (2009) ISBN:0702029793. Read it at Google Books - Find it at Amazon
- 4. Morimura T, Nakano A, Matsumoto T et-al. Chondromyxoid fibroma of the frontal bone. AJNR Am J Neuroradiol. 13 (4): 1261-4. AJNR Am J Neuroradiol (abstract) - Pubmed citation
- 5. Eisenberg RL. Bubbly lesions of bone. AJR Am J Roentgenol. 2009;193 (2): W79-94. doi:10.2214/AJR.09.2964 - Pubmed citation
- 6. Stacy GS, Peabody TD, Dixon LB. Mimics on radiography of giant cell tumor of bone. AJR Am J Roentgenol. 2003;181 (6): 1583-9. AJR Am J Roentgenol (full text) - Pubmed citation
- 7. Jaffe HL, Lichtenstein L. Chondromyxoid fibroma of bone; a distinctive benign tumor likely to be mistaken especially for chondrosarcoma. Arch Pathol (Chic). 2008;45 (4): 541-51. Pubmed citation
- 8. Budny AM, Ismail A, Osher L. Chondromyxoid fibroma. J Foot Ankle Surg. 47 (2): 153-9. doi:10.1053/j.jfas.2007.08.013 - Pubmed citation
- 9. Mehta S, Szklaruk J, Faria SC et-al. Radiologic-pathologic conferences of the University of Texas M. D. Anderson Cancer Center: Chondromyxoid fibroma of the sacrum and left iliac bone. AJR Am J Roentgenol. 2006;186 (2): 467-9. doi:10.2214/AJR.05.1108 - Pubmed citation
- 10. Desai SS, Jambhekar NA, Samanthray S et-al. Chondromyxoid fibromas: a study of 10 cases. J Surg Oncol. 2005;89 (1): 28-31. doi:10.1002/jso.20113 - Pubmed citation
- 11. Eybalin MC, Danais S, Marton D et-al. [Chondromyxoid fibroma: radiologic and radioisotope aspects]. Can Assoc Radiol J. 1988;39 (2): 148-51. - Pubmed citation
- 12. Unni KK, Inwards CY, Research MF. Dahlin's bone tumors, general aspects and data on 10,165 cases. Lippincott Williams & Wilkins. (2009) ISBN:0781762421. Read it at Google Books - Find it at Amazon
- 13. Layfield LJ. Cytopathology of bone and soft tissue tumors. Oxford University Press, USA. (2002) ISBN:019513236X. Read it at Google Books - Find it at Amazon
- 14. Hefti F, Brunner R, Hasler CC et-al. Pediatric Orthopedics in Practice. Springer Verlag. (2007) ISBN:3540699635. Read it at Google Books - Find it at Amazon
- 15. Wilson AJ, Kyriakos M, Ackerman LV. Chondromyxoid fibroma: radiographic appearance in 38 cases and in a review of the literature. Radiology. 1991;179 (2): 513-8. Radiology (abstract) - Pubmed citation
- 16. Macdonald D, Fornasier V, Holtby R. Chondromyxoid fibroma of the acromium with soft tissue extension. Skeletal Radiol. 2000;29 (3): 168-70. Pubmed citation
- 17. Beggs IG, Stoker DJ. Chondromyxoid fibroma of bone. Clin Radiol. 1983;33 (6): 671-9. Pubmed citation
- 18. Zillmer DA, Dorfman HD. Chondromyxoid fibroma of bone: thirty-six cases with clinicopathologic correlation. Hum. Pathol. 1989;20 (10): 952-64. Pubmed citation
- 19. Kim HS, Jee WH, Ryu KN et-al. MRI of chondromyxoid fibroma. Acta Radiol. 2011;52 (8): 875-80. Acta Radiol (full text) - doi:10.1258/ar.2011.110180 - Pubmed citation
- 20. Mitchell M, Sartoris DJ, Resnick D. Case report 713. Chondromyxoid fibroma of the third metatarsal. Skeletal Radiol. 1992;21 (4): 252-5. Pubmed citation
- 21. Kreicbergs A, LöNnquist PA, Willems J. Chondromyxoid fibroma. A review of the literature and a report on our own experience. Acta Pathol Microbiol Immunol Scand A. 1985;93 (4): 189-97. Pubmed citation