eMedicine Specialties > Orthopedic Surgery > Neoplasms

Chondromyxoid Fibroma

Author: Hannah D Morgan, MD, Consulting Staff, Connecticut Orthopaedic Specialists
Coauthor(s): Timothy A Damron, MD, David G Murray Endowed Professor, Department of Orthopedic Surgery, Professor, Orthopedic Oncology and Adult Reconstruction, Vice Chair, Department of Orthopedics, State University of New York Upstate Medical University at Syracuse
Contributor Information and Disclosures

Updated: Jan 18, 2008

Introduction

Background

Chondromyxoid fibroma (CMF) is a rare, slow-growing bone tumor of chondroblastic derivation.1,2,3,4 Jaffe and Lichtenstein first described the condition in 1943.5 They differentiated this benign lesion from chondrosarcoma, a much more common, but malignant, tumor.

See also the following topic in eMedicine:
Chondromyxoid Fibroma [Radiology]

Pathophysiology

Grossly, CMFs are firm, grayish-white masses that are sharply demarcated; they are lobulated or pseudolobulated. Their appearance can mimic fibrous tissue or hyaline cartilage. The lesions can destroy trabecular bone and may thin the cortex. Some CMFs may have areas of hemorrhage or cystic degeneration. A liquid, mucinous appearance may increase the suggestion of chondrosarcoma.

Many chondromyxoid fibromas display morphologic features that resemble different stages of chondrogenesis.6

A study by Romeo and colleagues examined the DNA microarray of chondromyxoid fibroma (as well as that of chondroblastoma).7 The authors found that the differential expression of adhesion and extracellular matrix molecules, including CD166, versican, perlecan, and Col4A2, may interfere with cartilaginous differentiation.

Frequency

United States

CMF accounts for less than 1% of primary bone tumors.

International

As of 2000, approximately 500 cases of CMF had been described in the world literature.8

Mortality/Morbidity

Local symptoms of CMF may cause a reduction in activity. CMF may recur locally, especially following a marginal excision. In addition, it may behave in an active or aggressive fashion, but malignant conversion is extremely rare and is difficult to distinguish from misdiagnosed de novo chondrosarcoma. Consequently, mortality from true benign CMF is essentially nonexistent.

Race

No racial predilection has been reported.

Sex

According to most reports, males and females are affected equally, although a few series have reported a male predominance.9

Age

CMF primarily affects young adults in their second and third decades of life. Eighty percent of patients are younger than 36 years. The youngest reported patient was aged 3 years at the time of diagnosis, and the oldest patient was aged 87 years at the time of diagnosis.

Clinical

History

Approximately 70% of patients have symptoms at the time of diagnosis. The remaining lesions are discovered incidentally. Pain is the most common symptom and may be present for years. While typically mild, the pain may become more severe with time, and night symptoms may be present. Patients may also report swelling and, in very rare cases, a limitation of joint motion.

Physical

Approximately 89% of CMFs involve the lower extremity. The proximal tibia is the most common location, followed by the distal femur, pelvis, and foot.10 Long bones are involved much more frequently than are other bones, especially in younger patients. Flat bone involvement may be observed more often in older patients. Patients may have localized tenderness or swelling over the CMF lesion, and, in rare cases, they may incur a pathologic fracture.

Causes

Although no specific cause is known for CMF, some authors have noted an association with certain chromosomal abnormalities. In a study of 4 patients with CMF, Granter and colleagues found that all of the subjects had a clonal rearrangement of chromosome 6.11 Each of these rearrangements involved band 6q13, which has not been associated with other bone tumors. Thus, band 6q13 may be useful as a cytogenetic marker to distinguish CMF from other histologically similar tumors. The authors suggested that oncogene activation resulting from this clonal rearrangement is likely to be involved in the genesis of CMF.

More on Chondromyxoid Fibroma

Overview: Chondromyxoid Fibroma
Differential Diagnoses & Workup: Chondromyxoid Fibroma
Treatment & Medication: Chondromyxoid Fibroma
Follow-up: Chondromyxoid Fibroma
Multimedia: Chondromyxoid Fibroma
References
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References

  1. McGrory BJ, Inwards CY, McLeod RA, et al. Chondromyxoid fibroma. Orthopedics. Mar 1995;18(3):307-10. [Medline].

  2. Ralph LL. Chondromyxoid fibroma of bone. J Bone and Joint Surg. 1962;44B(1):7-24.

  3. Schutt PG, Frost HM. Chondromyxoid fibroma. Clin Orthop Relat Res. 1971;78:323-9. [Medline].

  4. White PG, Saunders L, Orr W, et al. Chondromyxoid fibroma. Skeletal Radiol. Jan 1996;25(1):79-81. [Medline].

  5. Jaffe HL, Lichtenstein L. Chondromyxoid fibroma of bone: a distinctive benign tumor likely to be mistaken especially for chondrosarcoma. Arch Path. 1943;19:541-51.

  6. Baker AC, Rezeanu L, O'Laughlin S, et al. Juxtacortical chondromyxoid fibroma of bone: a unique variant: a case study of 20 patients. Am J Surg Pathol. Nov 2007;31(11):1662-8. [Medline].

  7. Romeo S, Oosting J, Rozeman LB, et al. The role of noncartilage-specific molecules in differentiation of cartilaginous tumors: lessons from chondroblastoma and chondromyxoid fibroma. Cancer. Jul 15 2007;110(2):385-94. [Medline].

  8. Blackwell JB, Norma M, Curnow P. Benign bone tumours in Western Australia, 1972-1996. Pathology. Dec 2007;39(6):567-74. [Medline].

  9. Wu CT, Inwards CY, O''Laughlin S, et al. Chondromyxoid fibroma of bone: a clinicopathologic review of 278 cases. Hum Pathol. May 1998;29(5):438-46. [Medline].

  10. Wu KK. Chondromyxoid fibroma of the foot bones. J Foot Ankle Surg. Sep-Oct 1995;34(5):513-9. [Medline].

  11. Granter SR, Renshaw AA, Kozakewich HP, et al. The pericentromeric inversion, inv (6)(p25q13), is a novel diagnostic marker in chondromyxoid fibroma. Mod Pathol. Nov 1998;11(11):1071-4. [Medline].

  12. Heydemann J, Gillespie R, Mancer K. Soft tissue recurrence of chondromyxoid fibroma. J Pediatr Orthop. Nov-Dec 1985;5(6):725-7. [Medline].

  13. Kyriakos M. Soft tissue implantation of chondromyxoid fibroma. Am J Surg Pathol. Aug 1979;3(4):363-72. [Medline].

  14. Dahlin DC. Chondromyxoid fibroma of bone, with emphasis on its morphological relationship to benign chondroblastoma. Cancer. Jan-Feb 1956;9(1):195-203. [Medline].

  15. Sakayama K, Sugawara Y, Kidani T, et al. Diagnostic and therapeutic problems of giant cell tumor in the proximal femur. Arch Orthop Trauma Surg. Dec 2007;127(10):867-72. [Medline].

  16. Marin C, Gallego C, Manjon P, et al. Juxtacortical chondromyxoid fibroma: imaging findings in three cases and a review of the literature. Skeletal Radiol. Nov 1997;26(11):642-9. [Medline].

  17. Merine D, Fishman EK, Rosengard A, et al. Chondromyxoid fibroma of the fibula. J Pediatr Orthop. Jul-Aug 1989;9(4):468-71. [Medline].

  18. Yamaguchi T, Dorfman HD. Radiographic and histologic patterns of calcification in chondromyxoid fibroma. Skeletal Radiol. Oct 1998;27(10):559-64. [Medline].

  19. Cabral CE, Romano S, Guedes P, et al. Chondromyxoid fibroma of the lumbar spine. Skeletal Radiol. Aug 1997;26(8):488-92. [Medline].

  20. Nielsen GP, Keel SB, Dickersin GR, et al. Chondromyxoid fibroma: a tumor showing myofibroblastic, myochondroblastic, and chondrocytic differentiation. Mod Pathol. May 1999;12(5):514-7. [Medline].

  21. Durr HR, Lienemann A, Nerlich A, et al. Chondromyxoid fibroma of bone. Arch Orthop Trauma Surg. 2000;120(1-2):42-7. [Medline].

  22. Hristov B, Shokek O, Frassica DA. The role of radiation treatment in the contemporary management of bone tumors. J Natl Compr Canc Netw. Apr 2007;5(4):456-66. [Medline].

  23. Gherlinzoni F, Rock M, Picci P. Chondromyxoid fibroma. The experience at the Istituto Ortopedico Rizzoli. J Bone Joint Surg Am. Feb 1983;65(2):198-204. [Medline].

  24. Mikulowski P, Ostberg G. Recurrent chondromyxoid fibroma. Acta Orthop Scand. 1971;42(5):385-90. [Medline].

  25. Zillmer DA, Dorfman HD. Chondromyxoid fibroma of bone: thirty-six cases with clinicopathologic correlation. Hum Pathol. Oct 1989;20(10):952-64. [Medline].

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Further Reading

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Keywords

CMF, bone tumor, chondroblast, chondrosarcoma, benign tumor, aneurysmal bone cyst

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Contributor Information and Disclosures

Author

Hannah D Morgan, MD, Consulting Staff, Connecticut Orthopaedic Specialists
Hannah D Morgan, MD is a member of the following medical societies: American Academy of Orthopaedic Surgeons and American Medical Association
Disclosure: Nothing to disclose.

Coauthor(s)

Timothy A Damron, MD, David G Murray Endowed Professor, Department of Orthopedic Surgery, Professor, Orthopedic Oncology and Adult Reconstruction, Vice Chair, Department of Orthopedics, State University of New York Upstate Medical University at Syracuse
Timothy A Damron, MD is a member of the following medical societies: American Academy of Orthopaedic Surgeons, American College of Surgeons, American Medical Association, Children's Oncology Group, Connective Tissue Oncology Society, Musculoskeletal Tumor Society, Orthopaedic Research Society, and Society for Experimental Biology and Medicine
Disclosure: Nothing to disclose.

Medical Editor

Howard A Chansky, MD, Associate Professor, Department of Orthopedics and Sports Medicine, University of Washington Medical Center
Howard A Chansky, MD is a member of the following medical societies: American Academy of Orthopaedic Surgeons
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

CME Editor

Dinesh Patel, MD, FACS, Associate Clinical Professor of Orthopedic Surgery, Harvard Medical School; Chief of Arthroscopic Surgery, Department of Orthopedic Surgery, Massachusetts General Hospital
Dinesh Patel, MD, FACS is a member of the following medical societies: American Academy of Orthopaedic Surgeons, American Association of Physicians of Indian Origin, American College of International Physicians, and American College of Surgeons
Disclosure: Nothing to disclose.

Chief Editor

Harris Gellman, MD, Consulting Surgeon, Broward Hand Center, Voluntary Clinical Professor of Orthopedic Surgery and Plastic Surgery, Departments of Orthopedic Surgery and Surgery, University of Miami School of Medicine
Harris Gellman, MD is a member of the following medical societies: American Academy of Medical Acupuncture, American Academy of Orthopaedic Surgeons, American Orthopaedic Association, American Society for Surgery of the Hand, and Arkansas Medical Society
Disclosure: Nothing to disclose.

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