CHILDREN WITH VESICAL RHABDOMYOSARCOMA (RMS) TREATED BY PARTIAL CYSTECTOMY WITH NEOADJUVANT OR ADJUVANT CHEMOTHERAPY, WITH OR WITHOUT RADIOTHERAPY - A REPORT FROM THE INTERGROUP RHABDOMYOSARCOMA STUDY (IRS) COMMITTEE

Purpose: Children with bladder rhabdomyosarcoma (RMS) are currently tr eated with primary chemotherapy and usually with local irradiation. Mo re than 30% of this group ultimately require total cystectomy. The pur pose of this study was to review the results of the use of partial as opposed to total cystectomy in 40 patients with bladder RMS during Int ergroup Rhabdomyosarcoma Study (IRS)-I, -II, and -III (1972-1989). Pat ients and Methods: A total of 171 children with primary RMS of the bla dder were enrolled in the IRS during this interval. Partial cystectomy was performed in 40, in 33 before any other therapy and in seven afte r 10-57 weeks of chemotherapy (primarily vincristine, actinomycin-D, a nd cyclophosphamide, i.e., VAC) +/- radiotherapy. Surviving patients h ave been observed for 4-22 years. Results: Thirty-one of 40 patients ( 78.5%) have been disease free for 2-16 years. Survival among all other IRS cases with bladder RMS during the same interval was 79.5%. Of the 31 surviving patients, one required secondary total cystectomy and tw o required bladder augmentation procedures for benign bladder contract ure. Three quarters of the total group of living patients who have und ergone partial cystectomy are without bladder-related symptoms or demo nstrable lower urinary tract disease. The remaining patients have a hi story of functional bladder problems related to contracture or inconti nence. Conclusions: Partial cystectomy is an alternative to total cyst ectomy for bladder RMS when the tumor site makes it anatomically feasi ble, In such patients, it should be considered before total cystectomy and in patients with persistent areas of questionable residual tumor after otherwise apparently successful regimens of primary chemotherapy +/- radiotherapy.