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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.