dr james c wittig, orthopedic oncologist, new york, new jersey
About Dr. Wittig
Mesenchymal Chondrosarcoma

General Information

  • High grade malignant, cartilage-forming tumor
  • Comprised primarily of noncartilaginous small, round, oval, or spindle shaped cells with islands of malignant cartilage dispersed throughout noncartilaginous component of tumor
  • Osteoid may be present as well
  • Tumor frequently has a hemangiopericytoma-like appearance
  • Metastasizes to the lungs and lymph nodes
  • May have chondroid matrix calcification

Clinical Presentation

Signs/Symptoms:

  • Pain and occasionally swelling
  • ~ 1/3 of patients are symptomatic for more than 1 year

Prevalence:

  • ~2% of all chondrosarcomas
  • No sexual or racial predilection

Age:

  • All ages
  • Predominantly affects those between the ages of 10 and 40

Sites:

  • Arises in bone and soft tissue (1/3 of cases arise from soft tissue)
  • Favors the femur, ribs, spine, maxilla, mandible, and pelvis
  • Other long tubular bones and phalanges may also be affected

Radiographic Presentation

  • Aggressive motheaten to permeative lesion
  • Indistinct border in most cases
  • Osseous destruction with a soft tissue component
  • Chondroid matrix calcification may be present (60-70% of cases)
  • Soft tissue mass
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Plain X-ray: Mesenchymal Chondrosarcoma from Proximal Humerus Plain X-ray: Mesenchymal Chondrosarcoma from Proximal Humerus
Plain Radiograph of an Extraskeletal Mesenchymal Chondrosarcoma Plain X-ray: Extraskeletal Mesenchymal Chondrosarcoma of Popliteal Fossa
MRI: Extraskeletal Mesenchymal Chondrosarcoma of Popliteal Fossa MRI: Extraskeletal Mesenchymal Chondrosarcoma of Popliteal Fossa
MRI with Gadolinium: Extraskeletal Mesenchymal Chondrosarcoma Extensive Necrosis Demonstrated indicative of High Grade Tumor MRI: Extraskeletal Mesenchymal Chondrosarcoma
MRI: Extraskeletal Mesenchymal Chondrosarcoma CT Scan: Extraskeletal Mesenchymal Chondrosarcoma of Popliteal Fossa No Mineralization Detected

Gross Pathology

  • Gross appearance is variable
  • Ranging from soft to firm
  • Gray to pink
  • Occasionally have a faintly lobulated pattern
  • Grossly obvious cartilage is rarely visualized
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Mesenchymal Chondrosarcoma, gross pathology Mesenchymal Chondrosarcoma, gross pathology Mesenchymal Chondrosarcoma, gross pathology

Microscopic Pathology

  • Neoplastic cells may be small, round, oval, or spindle shaped
    • Undifferentiated mesenchymal cells similar to Ewing sarcoma
  • Low grade islands of cartilage scattered throughout the mesenchymal cells
    • Usually only a small part of lesion
    • Cytologically low grade
    • Usually sharply demarcated from surrounding stroma
    • Stain S-100 positive
    • Cells within cartilage
    • Tend to have round or ovoid nuclei
    • Lacunae are poorly formed
  • May contain islands of collagen resembling osteoid
  • Lesions are vascular and often have large, anastomosing vessels that impart hemangiopericytoma-like pattern
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Mesenchymal Chondrosarcoma, microscopic pathology Mesenchymal Chondrosarcoma, microscopic pathology
Mesenchymal Chondrosarcoma, microscopic pathology Cartilage Component, Mesenchymal Component
Mesenchymal (Small Round Blue Cell) Component Large Nuclei; No Cytoplasm; No Matrix Mesenchymal (Small Round Blue Cell) Component Hemagiopericytoma-like Pattern of Blood Vessels
Large Staghorn Blood Vessels

Differential Diagnosis

  • Ewing Sarcoma
  • Small Cell Osteosarcoma
  • Dedifferentiated Chondrosarcoma

Biological Behavior

  • Locally aggressive
    • Cortical destruction in approximately half of cases
    • Extension of tumor into adjacent soft tissues
  • High metastatic and local recurrence rates
    • Metastasizes primarily to lungs, other bones, lymph nodes and viscera
    • Metastases may not appear for over 5 years after treatment
    • Over 70% mortality

Treatment & Prognosis

  • Most patients are treated with a combination of surgery and chemotherapy. Radiation is used in selected cases, particularly extraskeletal mesenchymal chondrosarcomas
  • Wide/Radical limb sparing surgery whenever feasible (most cases)
  • Amputation for very large or unresectable tumors
Roll over the images for more information
Limb Sparing Surgery for Extraskeletal Mesenchymal Chondrosarcoma of Popliteal Fossa Tumor
Portion of Popliteal Artery Removed with Tumor, Sciatic Nerve Popliteal Artery Re-anastomosed
Specimen Limb Sparing Surgery for Extraskeletal Mesenchymal Chondrosarcoma of Popliteal Fossa Intraoperative Arteriogram demonstrating Patency of Popliteal Artery at Conclusion of Surgery
Limb Sparing Surgery for Extraskeletal Mesenchymal Chondrosarcoma of Popliteal Fossa Rotational Sartorius Muscle Flap for Closure
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