dr james c wittig, orthopedic oncologist, new york, new jersey
About Dr. Wittig
Chondromyxofibroma (CMF)

General Information

Dedifferentiated chondrosarcoma consists of a low grade malignant hyaline cartilage tumor associated with a high-grade nonchondroid spindle sarcoma. The two components are juxtaposed with abrupt clear demarcation line

  • The high grade sarcoma is most commonly an MFH, osteosarcoma or fibrosarcoma although others may occur
  • It is an extremely aggressive tumor with a high metastatic rate and dismal prognosis
  • Constitutes approximately 10% of chondrosarcomas
  • 50% arise from a secondary chondrosarcoma

Clinical Presentation

Signs/Symptoms:

  • Pain, with or without pathological fracture
  • Swelling
  • Parasthesias
  • Symptoms usually last around 6-10 months

Prevalence:

  • 11% of all chondrosarcomas
  • No predilection for sex or race

Age:

  • Young adulthood to old age
  • Most patients are older than 50

Sites:

  • Most common in pelvis, proximal femur, proximal humerus, distal femur, ribs
  • Similar sites as conventional chondrosarcoma

Radiographic Presentation

Radiology emulates pathology: Biphasic Tumor

  • One region low grade chondrosarcoma
  • Second m

    ore aggressive area with bone destruction, lysis of calcification, soft tissue mass

  • Cortical permeation and a soft tissue mass in 70% of cases

Ill-defined, lytic intraosseous lesion

  • Or extraosseous soft tissue mass
    • Devoid of calcifications in continuity with lesions having the features of a cartilaginous tumor

Characteristically abrupt transition between chondroid tumor and dedifferentiated, lytic component

Bone may be expanded and adjacent cortex thickened

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Gross Pathology

  • Areas with typical lobular, blue-gray myxoid hyaline cartilage tissue
    • Or overtly cartilaginous appearance of mature hyaline cartilage
  • Zones of brown, tan, or hemorrhagic tissue
    • Lacking the consistency of cartilage
  • Dedifferentiated component may only be a minor portion of the overall tumor
  • May also be so extensive that little cartilage is recognizable
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Microscopic Pathology

  • Chondrosarcoma component is often grade I (Low Grade Hyaline Type Cartilage)
  • Dedifferentiated component
    • Predominant noncartilaginous/spindle sarcoma component varies
      • Fibrosarcoma and MFH most frequently reported
        • MFH is a high grade pleomorphic spindle cell tumor with a storiform pattern
    • Osteosarcoma is third most common dedifferentiated component
    • Rhabdomyosarcoma and angiosarcoma also reported

Junction of cartilaginous and noncartilaginous components is sharp and distinct. There are no dedifferentiated areas admixed in the middle of the cartilaginous areas

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Differential Diagnosis

  • High Grade Chondrosarcoma with Spindle Cell Areas
  • Mesenchymal Chondrosarcoma
  • Chondroblastic Osteosarcoma
  • Malignant Fibrous Histiocytoma
  • Fibrosarcoma

Biological Behavior

  • Very aggressive locally
    • Frequently cortical perforation
    • Mass is usually large if extraosseous extension occurs
  • Extremely high metastatic rate
    • Metastasizes primarily to lungs
    • Also bones and other organs

Treatment & Prognosis

  • Wide/Radical limb sparing resection whenever feasible
  • Amputation may be necessary for large tumors
  • Chemotherapy may be considered for high grade dedifferentiated component but is controversial and no clear cut benefit has ever been demonstrated
  • Radiation may be considered if the tumor is unresectable or if a wide margin can not be achieved with surgery such as with large pelvic tumors. Radiation would be used as an adjuvant to eradicate any residual microscopic disease.
  • Prognosis almost hopeless, regardless of extent of resection
    • 90% of patients are dead of metastatic disease within 2 years
      • Most of these die within 1 year
    • Metastases consist solely of high-grade dedifferentiated component
      • Predominantly in lungs
      • Also bones and other organs
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