Introduction: Soft-tissue sarcomas (STS) in children and young adults are rare. This is a heterogeneous group of tumors, which is traditionally divided to rhabdomyo-sarcomas and non-rhabdomyosarcoma soft-tissue sarcomas (NRSTS). These tumors are further classified to high- and low-grade tumors.

Material and Methods: Between 1988 and 1999, the authors treated 50 patients (25 males, 25 females) under the age of 20 who were diagnosed with a soft-tissue sarcoma.

Histopathological Diagnoses: rhabdomyosarcoma – 11, synovial sarcoma – 6, other high-grade STS (extraskeletal Ewing’s sarcoma, epitheloid sarcoma, neurofibrosarcoma, hemangiopericytoma, fibrosarcoma, and unclassified sarcoma) – 17. Seven patients were diagnosed with low-grade STS and 9 patients with an aggressive desmoid tumor.

Anatomic Location: Lower extremities – 30, upper extremities – 9, shoulder girdle – 2, trunk – 4, pelvic girdle – 5.

Preoperative Treatment: Thirty patients received neo-adjuvant chemotherapy, four patients underwent isolated limb perfusion with TNF and melphalan, and one patient received preoperative radiation therapy. Surgery: Forty-seven underwent limb-sparing resections and 3 underwent primary amputation. Wide margins were achieved in 37 patients and marginal margins in 10. Intralesional resection was performed in 3 patients.

Postoperative Treatment: Thirty-seven patients received adjuvant chemotherapy and 34 received radiation therapy.

Oncological Status: At the most recent follow-up, 24 patients of the 37 patients with high-grade STS have no evidence of disease, three are alive with disease, and seven are dead. Fourteen of the 16 patients with low-grade tumors have no evidence of disease and 2 are alive with disease. There were 4 secondary amputations due to local tumor recurrence.

Conclusions: Management of soft-tissue sarcomas in children and young adults requires the judgmental use of pre- and postoperative treatment modalities. Local tumor control can be achieved in the majority of the patients. A longer follow-up is required to determine the overall survival of these patients.

Background: The surgical treatment of extensive diffuse Pigmented Villonodular Synovitis (PVNS) of large joints alone, is unsatisfactory, with high rates of local recurrence. Postsynovectomy adjuvant treatment with external beam radiation therapy or intraarticular injection of Yttrium90 (Y90) yielded better results.

Aims: Experience with 10 cases treated with debulking surgery followed by intraarticular injection of Y90 is reported.

Methods: Between January 1989 and June 1998, 10 patients (8 males and 2 females aged 15049 years) with extensive diffuse PVNS were treated. In 6 patients the knee joint, in 3 patients the ankle joint, and in 1 patient the hip joint were involved. The 10 patients underwent 15 operations, 1 patient had 3 surgical procedures, and 3 patients underwent 2 surgeries (interval between re-operations for local recurrence were 2–4 years). All patients had an intraarticular injection of 15–25 mCi of Y90, 6–8 weeks after the last surgery.

Results: Follow up time was 2.5–12 years (mean 6 years). All patients were followed by repeated computerized tomography (CT) scans, magnetic resonance imaging (MRI), plain X-ray films and bone scans semi-annually. In 9 patients no evidence of disease and no progression of bone or articular destruction have been noted. In 1 patient stabilization of disease was achieved with no further evidence of bony or articular damage. No complications were noticed after surgery, nor after the intraarticular Y90 injection.

Conclusions: A combination of debulking surgery with intraarticular injection of Y90 for extensive diffuse PVNS of major joints is a reliable way of treatment with good results.