Surgery is the most important modality for local control in ES. If surgery is not possible alone to resect the primary tumor with negative margins, then radiation therapy (RT) should be used either pre- or post-operatively to ensure local control.
RT is associated with an increased risk of a second malignant neoplasm (often an osteogenic sarcoma) within the RT field many years after treatment and therefore should be avoided if possible.
Surgery used alone without chemotherapy is associated with survival rates of much less than 10%. Failure is usually due to distant metastases.
Chemotherapy is essential to reduce the bulk of primary disease initially and to treat metastases. However it is not possible to ablate all disease with chemotherapy alone – so surgery or RT or a combination of the two is essential to control local bulky disease and maintain function.
In the great majority of cases limb-sparing surgery is possible and amputation can be avoided.
Limb Sparing Surgery:
Goal |
- Remove the entire tumor while preserving margins of normal tissue all around the tumor
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Timing of Surgery |
- Performed after pre-operative chemotherapy
- Chemotherapy usually makes surgery possible
- Imaging studies must be repeated just prior to surgery to look at extent of disease
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Disadvantages |
- May not achieve complete local control
|
Indications |
- Tumor is relatively small
- There is no unmanageable pathologic fracture
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Rarely amputation may be necessary for ES.
Amputation:
Goal |
- Achieve complete local control of tumor
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Timing of Surgery |
- After pre-operative chemotherapy
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Disadvantages |
|
Indications |
- Tumor large and unresponsive to chemotherapy
- Presence of an unmanageable pathologic fracture
- RT would encompass the entire circumference of the limb and lead to significant complications (lymphoedema and vascular compromise)
Note: Many fractures heal during initial chemotherapy,
allowing for subsequent RT
|
Surgical Approach by Site:
Pelvis |
- Often associated with a large extraosseus component
- 3 areas of pelvis, each site may be resected independently:
- ilium
- periacetabulum
- pubic rami
- Many pelvic tumors involve iliac wing and can be resected with minimal functional loss
- Tumor involvement of the sacrum or acetabulum make resection less feasible
- Reconstruction less complex than at other sites
|
Shoulder |
- Determine length of bone and extraosseus component
- Intra-articular resection can be performed
- Reconstruction by prosthesis
|
Knee |
- Similar surgery to osteosarcoma of the knee joint
- Attempt to preserve distal femoral and proximal tibial epiphyses (major growth centers of bones)
|
Distal Femur |
- Goal: remove involved bone while maintaining good knee function
- Most of the quadriceps muscles are preserved
- Custom prosthesis used to replace the resected bone
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Proximal Tibia |
- Higher morbidity, more difficult than distal femur
- Often need to ligate the anterior tibial vessels
- Reconstruction by endoprosthesis or an osteoarticular allograft
|
Fibula |
- Middle and proximal fibula are expendable bones
- Usually tumors are large and extend into the popliteal space
- Resection usually includes removal of the fibula, adjacent peroneal muscles, and a portion of the anterior compartment
- Major surgical decision: preserve or sacrifice peroneal nerve:
- Usually sacrificed
- Result is drop foot
- Usually lateral malleolus must be preserved
|
Spine |
- Most tumors associated with paravertebral and/or epidural extension
- Patients often present with neurologic signs and require rapid therapeutic decisions
- Initial diagnosis via needle biopsy
|
Chest Wall/Ribs |
- Removal of entire rib and adjacent soft tissue or a portion of adjacent lung
- 1-4 ribs often need to be removed for a complete resection
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