Updated: 6/21/2021

Chondromyxoid Fibroma

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https://upload.orthobullets.com/topic/8022/images/Case D - tibia - xray b -Parsons_moved.png
https://upload.orthobullets.com/topic/8022/images/mricmfcoronal.jpg
https://upload.orthobullets.com/topic/8022/images/Histology A - Stellate and giant cells - Parsons_moved.png
https://upload.orthobullets.com/topic/8022/images/mricmfaxial.jpg
https://upload.orthobullets.com/topic/8022/images/bonescancmf.jpg
  • summary
    • Chondromyxoid Fibromas are rare, benign, chondrogenic lesions characterized by variable amounts of chondroid, fibromatoid, and myxoid elements that are most commonly found in the metaphysis of long bones. Patients typically present between the ages of 10 and 30 with regional pain and swelling. 
    • Diagnosis is made with biopsy showing hypercellular area with lobules of fibromyxoid tissue and myxoid stroma with stellate cells.
    • Treatment is usually intralesional curettage and bone grafting (or PMMA).
  • Epidemiology
    • Demographics
      • more common in males
      • most common in second and third decades of life
        • may affect patients up to 75 years old
    • Anatomic location
      • long bones (ie. tibia, distal femur)
        • often affects metaphyseal (proximal tibia) regions
      • pelvis
      • feet or hands
  • Etiology
    • Pathophysiology
      • may arise from physeal remnants
    • Genetics
      • mutations
        • a genetic rearrangement may affect chromosome 6 (postion q13)
  • Presentation
    • History
      • long standing pain (months to years)
      • may be incidentally identified
    • Symptoms
      • pain and mild swelling
  • Imaging
    • Radiographs
      • findings
        • lytic, eccentric metaphyseal lesion
          • sharply demarcated from adjacent bone
          • scalloped and sclerotic rim
          • calcifications are rare
          • cortical expansion may be seen
          • lesion size may range from two to ten centimeters
    • MRI
      • findings
        • low signal on T1-weighted images
        • high signal on T2-weighted images
    • Bone scan
      • findings
        • increased signal uptake will be seen
  • Studies
    • Histology
      • findings
        • low-power
          • biphasic appearance
            • hypercellular area with lobules of fibromyxoid tissue
              • spindle-shaped cells or stellate-shaped cells
                • the cells contain hyperchromatic nuclei
                • multinucleated giant cells and fibrovascular tissue are located between lobules
            • hypocellular area with chondroid material
        • high power
          • myxoid stroma with stellate cells
          • regions of pleiomorphic cells with bizarre nuclei may be seen
    • Diagnostic criteria
      • histopathologic examination is mandatory for confirmation of the diagnosis
  • Differential
    • Radiographic
      • aneurysmal bone cyst (ABC)
      • chondroblastoma
      • non-ossifying fibroma
    • Histologic
      • chondroblastoma
      • enchondroma
      • chondrosarcoma
  • Treatment
    • Operative
      • intralesional curretage and bone grafting (or PMMA)
        • indications
          • mainstay of treatment
  • Complications
    • Recurrence
      • occurs in 25% of cases
  • Prognosis
    • Natural history
      • recurrence in CMF is not uncommon
        • may occur in 20-30% of cases
    • Negative prognostic variables
      • children
      • tumor is more lobulated with abundant myxoid material
    • Metastasis
      • has not been reported
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Questions (4)

(OBQ12.133) A 12-year-old female presents for evaluation of worsening right wrist pain. She denies any trauma and is otherwise healthy. A current radiograph is shown in Figures A and B. A biopsy is obtained and shown in Figure C. What is the most likely diagnosis?

QID: 4493
FIGURES:
1

Chondrosarcoma

5%

(213/4364)

2

Osteosarcoma

2%

(91/4364)

3

Chondromyxoid fibroma

49%

(2148/4364)

4

Nonossifying fibroma

33%

(1432/4364)

5

Enchondroma

10%

(452/4364)

L 4 C

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