Klin Onkol 1998; 11(4): 116-119.
Summary: Soft tissue sarcomas are the third commonest solid tumors of childhood. Approximately 70% of them arc rhabdomyosarcomas and undifferentiated sarcomas. The prognosis of patients with localized disease has improved in the last 20 years with a 5 year survival rale of 65%. Palients have been treated by chemotherapy in combination with surgery or radiotherapy. An unresolved issue is the management of patients with primary disseminated minors where three years survival rates are between 20-30%. From January 1992 to December 1996, thirty-five of 55 patients aged from 0.3 - 18.3'years entered ihe study. Distribution inlo ihe clinical stages; stage l.-10pts, stage II.-16pts, stage lll.-3pts. stage IV.-6pts. Predominant histopathological subclassifications were embryonal (13pts) and alveolar (11 pts) variants of RMS. The most frequent sites were extremities and trunk (14pts), genitourinary tract (8pts), head and neck parameningeal (7pts) and non- parameningeal (6pts) regions. We subdivided patients into the 5 treatment groups according to risk factors. With a median follow up 3.4 years, overall survival rates (OS) were 66% and event free survival rates (EFS) 54%. In stage I, OS and EFS were 90% and 60%, stage II. 69% and 63%, stage III. and IV. 33% and 33%. Patients wiih embryonal and botryoid RMS (EFS 77%, 75%) in non-parameningeal head and neck region did significantly improve compared to the alveolar valiant and extremities and trunk regions (EPS 29%). In summary, it can be said thai our results seemed to be comparable lo SIOP studies. In comparison to an IRS III study, the EFS is (for a good risk group) slightly lower due to reduction of radiotherapy and chemotherapy. A treatment method leading to a better prognosis in primary disseminated or relapsed sarcomas needs to be identified.
