[Soft tissue sarcoma of the bladder and prostate. A report of the Polish Paediatric Solid Tumour Group (PPSTG)]

Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma in children younger then 15 years of age. The treatment of RMS localized in bladder or prostate still remains controversial. The aim of this study was analysis of treatment results in children with soft tissue sarcoma of bladder and prostate. From 1993 to 2001 the PPSTG has used three protocols to treat soft tissue sarcomas in children. The CWS-91 regimen has been served to treat stage I-III and SIOP-IV Intergroup study in stage IV. Since 1996 the CWS-96 protocol for all patients in stage I-IV has been used. After biopsy confirmation of the diagnosis patients were treated with chemotherapy and subsequent surgery. Radiotherapy was administrated with total dose 32Gy v 44.5Gy v 48Gy. The group of 19 patients aged from 3 months to 18 years with median age 6 y 4 m were evaluated. The median follow-up time was 42 months. RMS-embryonal was diagnosed in 11 patients, RMS-alveolare in 4 and others types in 4. Sixteen patients presented clinical stage III and 3 stage IV The tumours were primarily localised in bladder in 16 patients and prostate in 3. Eight were tumours of volume bigger than 10 cm. After induction chemotherapy two patients received partial cystectomy, 5 complete cystectomy and 3 complete cystectomy with genitourinary reconstructive. Eight patients did not receive surgery. The most common treatment failure was isolated, local relapse in 8 children particularly in patients with any second surgery. The EFS for all patients was 52% and for patients with localised RMS tumours 65%. Significant prognostic factors are the initial tumours volume, the lymph nodes infiltration and the response to the first chemotherapy cycle. Surgery is the most important procedure in local control of soft tissue sarcoma. Reconstructive options in poor and non responder patients can improve the EFS and life quality of those patients.