Introduction: The EUROpean Bone Over 40 Sarcoma Study (EURO-B.O.S.S.) was the first prospective internationalstudy for patients 41-65 years old with high-grade bone sarcoma treated with an intensive chemotherapy regimen derived from protocols for younger patients with high-grade skeletal osteosarcoma. Methods: Chemotherapy based on doxorubicin, cisplatin, ifosfamide, and methotrexate was suggested, but patients treated with other regimens at the investigators' choice were also eligible for the study. Results: The present report focuses on the subgroup of 218 patients with primary high-grade osteosarcoma. With a median follow-up of 47 months, the 5-year probability of overall survival (OS) was 66% in patients with localized disease and 22% in case of synchronous metastases. The 5-year OS in patients with localized disease was 29% in pelvic tumors, and 70% and 73% for extremity or craniofacial locations, respectively. In primary chemotherapy, tumor necrosis ≥90% was reported in 21% of the patients. There were no toxic deaths; however, hematological toxicity was considerable with 32% of patients experiencing 1 or more episodes of neutropenic fever. The incidence of nephrotoxicity and neurotoxicity (mainly peripheral) was 28% and 24%, respectively. After methotrexate, 23% of patients experienced delayed excretion, in 4 cases with nephrotoxicity.

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The use of isolated, hyperthermic limb perfusion (ILP) for locally recurrent synovial sarcoma in two children is reported. In both cases recurrence was biopsy-proven after multiple surgical excisions followed by adjuvant chemo- and radiotherapy while no systemic metastases could be detected. ILP was performed using high-dose recombinant tumor necrosis factor alpha (rhTNF alpha) combined with melphalan via an extracorporeal circulation. Six weeks later, the former tumor bed and irradiation field was excised together with myocutaneous flap reconstruction for soft tissue coverage. The resection specimen revealed extensive necrosis of the tumor in both cases. Six months after completion of treatment, near-perfect limb function was determined by general clinical assessment and validated scoring systems. ILP, an accepted technique in treating adult extremity sarcoma, might also be of value in children as a means of limb-saving therapy.

With a combined modality treatment, it is possible to achieve excellent local control rates and long-term survival in patients with synovial sarcoma of the extremities. Neoadjuvant treatment can enable limb-sparing surgery in most patients, even if the tumors are located in anatomically difficult areas.