Treatment Efficiency, Outcome and Surgical Treatment Problems in Patients Suffering from Bladder- / Prostate Rhabdomyosarcoma – A Report from the Cooperative Soft Tissue Sarcoma Trial CWS-96

Abstract

Purpose To analyze the clinical course, treatment modalities, complications and outcome of patients suffering from bladder-/prostate rhabdomyosarcoma (BPRMS) treated within the CWS 96 trial. Material and Methods 85 children with BPRMS were included in the trial. 63 patients with embryonal RMS were analyzed. Patients received 3 cycles of neoadjuvant chemotherapy. At week 9, reassessment using CT- or MRI scan was carried out. Depending on tumor size, age, and response, local therapy consisting of radiotherapy and / or surgery was initiated. After local therapy, adjuvant systemic therapy was continued. Results Patient's age ranged from 01 to 197 months. Median follow up was 63.3 months ± 27.8. 89% of the patients had advanced stage neoplasm (IRS-stage III: n = 56). The 5-year-OS for the whole group were 76.3% ± 5.6 and the 5-year-EFS 69.8% ± 6.2. 17 patients underwent preoperative radio-chemotherapy followed by tumor resection (5-y-OS: 87.1% ± 8.6). Eight patients were treated with solely radiochemotherapy (OS: 88.9% ± 10.5). 25 patients received preoperative chemotherapy followed by tumor resection (OS: 83.6% ± 7.5). 13 patients underwent incomplete tumor resection and were treated with radiotherapy postoperatively (OS: 39.9% ± 14.8, p Conclusions The choice of local therapy is an important factor for prognosis in these patients. Primary or secondary inadequate surgery compromises the outcome and should be avoided. Radiotherapy alone, complete surgical tumor resection or combined preoperative radiotherapy with surgical resection led to the same good local control rate and prognosis.