Conservative surgery in multimodal therapy for pelvic rhabdomyosarcoma in children

Abstract

SmminaryTwenty-six previously untreated children, median age 3.4 years, with pelvic rhabdomyosarcoma (RMS) were seen between 1983 and 1988. Fourteen were girls. The planned strategy was to conserve pelvic organs, especially the bladder, by using primary chemotherapy, conservative surgery and, in most cases, radiotherapy. With a median follow-up of 71 months (range 34-103 months) overall survival was 73%, with no treatment-related death. The bladder salvage rate of 88% in survivors with bladder base,prostate primaries was much higher than that reported by the United States Intergoup Rhabdomyosarcoma Studies (IRS), though many of the preserved bladders did not function normally. We identified problems with both radiological and histological off-treatment monitoring. The overall accuracy of computerised tomographic (CT') scanning for prediction of tumour recurrence was only 81%, and endoscopic biopsies proved misleading in four of the ten bladder base/prostate patients monitored by serial cystoscopy. We conclude that a higher cure rate can be achieved by using intensive chemotherapy/radiotherapy and conservative surgery to treat children with pelvic RMS. Factors that might contribute to our favourable bladder salvage results, compared with those of the IRS, include (a) the fact that one of two specialist surgeons monitored and operated on all these patients and (b) our increasing awareness, during the study, that post-chemotherapy, radiotherapy histopathology and pelvic CT scan appearances may be misleading. Referral to paediatric centres with special experience of pelvic RMS may help raise the rate of bladder salvage in these children.

Rare Malign Tumors of Bladder: Review of the Literature

“…Beş yıllık sağkalım oranları, hastalığın ilk tanı anında yaygın yayılım yoksa %82'dir. 8 Morbidite genellikle radyoterapi sonrası görülmektedir. Relaps genellikle tedavi sonrası ilk beş yılda görül-mekte ve salvaj kemoterapi ile tedavi edilmektedir.…”

Rhabdomyosarcoma of the bladder, prostate or vagina: the role of surgery

“…Combinations of polychemotherapy, surgery and radiotherapy have given survival rates as high as 85% in patients with localized disease [1][2][3][4]. Secondary to the evidence that RMS is sensitive to chemotherapy, organ-sparing treatment has been advocated in the last two decades, to achieve a better quality of life in these children [5][6][7][8]; however, late relapse has been reported [6]. In different series, bladder function after treatment, especially…”

Treatment modalities of bladder/prostate rhabdomyosarcoma: a review

“…All used some sort of combination CTX, usually VAC, and surgery with or without RT on most of their populations. 15,19,[47][48][49][50][51][52][53][54][55][56][57] Two of the studies 49,55 were primary surgery studies, while the remainder were primary CTX studies. Some of the primary CTX studies would base subsequent use of either surgery and/or RT on the initial CTX response.…”

“…Some of the primary CTX studies would base subsequent use of either surgery and/or RT on the initial CTX response. 15,47,54 Most of the studies with better OS and bladder salvage have less than 5 y follow-up. 19,49,52,54 Some of the studies showing poor long-term organ salvage had irregular use of RT.…”

“…15,47,54 Most of the studies with better OS and bladder salvage have less than 5 y follow-up. 19,49,52,54 Some of the studies showing poor long-term organ salvage had irregular use of RT. 53,56,57 Others with poor long-term salvage would 13 8 (62) 8 (62) 13 (100) 8 (62) N: population; OS: overall survival; DFS: disease-free survival; U: unreported.…”