1) Ewing's Sarcoma and Other Small Round Blue Cell Tumors:

• In the group of tumours often referred to as "round cell" or "blue cell" tumours (Ewing's sarcoma, primitive neuroectodermal tumor, Askin's tumor, histiocytic lymphoma) a combination of chemotherapy and radiotherapy is recommended.
• Ewing's sarcoma, relatively rare in the adult age group, is more often treated in the Paediatric setting.. Treatment of the adult population affected by these tumours generally parallels that recommended by the BC Children's' Hospital group.
• A program consisting of alternating combinations of drugs both before and after local control measures (surgery and /or radiation therapy).
• The role of surgery in the management of Ewing's sarcoma is still being defined except in cases of primary tumor involvement of an expendable bone where surgery avoids the use of radiation therapy(e.g. ribs or head fibula).
• Debulking surgery is under considered for tumors in sites not amenable to complete resection.
• Patients with metastatic Ewings are treated with curative intent as these tumors are very sensitive to chemotherapy. Occasionally high dose chemotherapy with total body radiation therapy and stem cell transplant may be offered.

2) Osteosarcoma /Malignant Fibrous Histiocytoma of Bone (MFH of Bone) / other High Grade or Spindle Cell Sarcomas of Bone

Adjuvant chemotherapy has now been established as an essential part of the management of osteogenic sarcoma. Randomized studies show a significantly improved overall survival for non-metastatic osteosarcoma patients treated with adjuvant chemotherapy (Link, Eilber). Spindle cell tumors of bone are also believed to benefit from adjuvant chemotherapy and are generally managed in the same osteosarcoma.

Once the staging studies have been completed and a biopsy diagnosis made, preoperative administration of a multi-drug program is preferred.

Lack of tumor necrosis seen in the final operative specimen in some instances may result in a change in the recommended post-operative regimen.

Patients with operable pulmonary metastatic disease at diagnosis or subsequently may be cured and therefore are treated aggressively with thoracotomy with or without chemotherapy.

3) Rhabdomyosarcomas

Rhabdomyosarcomas are tumors which occur primarily in the Pediatric age patient.

The primary management involves neoadjuvant chemotherapy, followed by surgery and radiation therapy.

In the adult setting, the protocols used are derived from the Paediatric group recommendations.

Adult patients are often treated in a manner similar to Ewings/PNET patients.

4) Adjuvant Chemotherapy for Other Soft Tissue Sarcomas

The role for routine adjuvant chemotherapy for soft tissue sarcoma patients remains controversial, but we recommend its discussion with patients in the following situations:

• large primitive tumours
• poor prognosis, high grade histologic subtypes that have been shown to be chemo-responsive, e.g. cellular myxoid liposarcoma
• marginal local excision cases in which radiation cannot be delivered
• bulky locally advanced disease with preoperative radiation therapy
• in the setting of a controlled randomized trial.