Characteristics and outcome of children with primary soft tissue sarcomas of extremities.

Objectives: To determine the characteristics and outcome or patients with primary soft tissue sarcomas of extremities in children.

Material And Methods: Thirty-six patients treated for soft tissues sarcomas were enrolled into the study. Features analysed: the incidence of soft tissues sarcoma of extremities, the time from first clinical symptoms to making the diagnosis, the primary site of tumour; histopathologic type of tumour, stage of disease, methods and results of the treatment.

Results: The time From first symptoms to making the diagnosis was 5.4 months (mean). The site of the tumour was the femur in 6 patients, arm in 3, knee in 1. Histopathologic types: synovial sarcoma in 4 patients, malignant haemangiopericytoma in 2, rhabdomyosarcoma in 2, sarcoma myogenes in 1, primitive neuroectodermal tumour in l. Stage of disease: III deg. -- 8 patients, IV deg. -- 2. Patients underwent treatment according to the soft tissue sarcoma protocols. Results of treatment: first complete remission was observed in 7 patients; second complete remission in 1, one patient is on postoperative treatment. One patient died.

Conclusions: 1. Combined treatment achieves full remission in the majority of patients with soft tissues sarcomas localized within the limbs. 2. In patients with large tumours (>5 cm) the treatment should to be started with inductive chemotherapy, and the surgery should be postponed. 3. Early excision of the tumour should be considered in cases of small tumours (< 5 cm), when resection with wide margin of healthy tissues is possible, without deteriorating the function of the limb or cosmetic damage.