- Tumor Grade & Histology
- Parosteal favorable; telangiectatic unfavorable
- Disease Extent
- metastatic disease unfavorable
- Tumor Size / Site
- Age
- Response of the primary tumor to pre-operative chemotherapy: very powerful predictor
- > 80-90% necrosis favorable
Treatment: Multimodal OS - Surgery
- Chemotherapy
- control of micrometastases
- Radiation
Treatment: Surgery OS - Removal of all gross tumor with wide (>5cm) margins en bloc and biopsy site through normal tissue planes is required
- Type of surgical procedure depends on tumor location, size, extramedullary extent, presence of distant metastatic disease, age, skeletal development, and life-style preference
- Metastatic sites must also be resected
- If/when relapse occurs, retrieval therapy must include resection
- Surgery alone 15-25% 5 year survival
- With multiagent chemotherapy 55-68%
- No difference between adjuvant or neoadjuvant chemotherapy
- Those with >90% tumor necrosis and complete resection 80-85%
Treatment: Chemotherapy OS - Bulky disease is considered somewhat chemotherapy resistant
- Subclinical metastases are sensitive to chemotherapy
- Most active agents include
- adriamycin, cisplatinum, high-dose methotrexate, ifosfamide, etoposide
- Best # and schedule of chemotherapy unclear
- Role of intensification after local control unclear
- Immune modulators under study
- Role of adjuvant chemotherapy after thoracotomy for recurrent disease unclear
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