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

Daniel M. Hays, R. Beverly Raney, Moody D. Wharam, Eugene Wiener, Thom E. Lobe, Richard J. Andrassy, Walter Lawrence, Jean Johnston, Bruce Webber, Harold M. Maurer

Research output: Contribution to journalArticle

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Abstract

Purpose: Children with bladder rhabdomyosarcoma (RMS) are currently treated with primary chemotherapy and usually with local irradiation. More than 30% of this group ultimately require total cystectomy. The purpose 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 Intergroup Rhabdomyosarcoma Study (IRS)-I, -II, and -III (1972–1989). Patients and Methods: A total of 171 children with primary RMS of the bladder were enrolled in the IRS during this interval. Partial cystectomy was performed in 40, in 33 before any other therapy and in seven after 10–57 weeks of chemotherapy (primarily vincristine, actinomycin-D, and cyclophosphamide, i.e., VAC) radiotherapy. Surviving patients have 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 two required bladder augmentation procedures for benign bladder contracture. Three quarters of the total group of living patients who have undergone partial cystectomy are without bladder-related symptoms or demonstrable lower urinary tract disease. The remaining patients have a history of functional bladder problems related to contracture or incontinence. Conclusions: Partial cystectomy is an alternative to total cystectomy for bladder RMS when the tumor site makes it anatomically feasible. 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.

Original languageEnglish (US)
Pages (from-to)46-52
Number of pages7
JournalJournal of Pediatric Hematology/Oncology
Volume17
Issue number1
DOIs
StatePublished - Feb 1995

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Rhabdomyosarcoma
Cystectomy
Adjuvant Chemotherapy
Urinary Bladder
Radiotherapy
Contracture
Drug Therapy
Urologic Diseases
Residual Neoplasm
Dactinomycin
Vincristine
Cyclophosphamide
Survival

Keywords

  • Bladder
  • Cystectomy
  • Rhabdomyosarcoma

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Hematology
  • Oncology

Cite this

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. / Hays, Daniel M.; Raney, R. Beverly; Wharam, Moody D.; Wiener, Eugene; Lobe, Thom E.; Andrassy, Richard J.; Lawrence, Walter; Johnston, Jean; Webber, Bruce; Maurer, Harold M.

In: Journal of Pediatric Hematology/Oncology, Vol. 17, No. 1, 02.1995, p. 46-52.

Research output: Contribution to journalArticle

Hays, Daniel M. ; Raney, R. Beverly ; Wharam, Moody D. ; Wiener, Eugene ; Lobe, Thom E. ; Andrassy, Richard J. ; Lawrence, Walter ; Johnston, Jean ; Webber, Bruce ; Maurer, Harold M. / 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. In: Journal of Pediatric Hematology/Oncology. 1995 ; Vol. 17, No. 1. pp. 46-52.
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abstract = "Purpose: Children with bladder rhabdomyosarcoma (RMS) are currently treated with primary chemotherapy and usually with local irradiation. More than 30{\%} of this group ultimately require total cystectomy. The purpose 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 Intergroup Rhabdomyosarcoma Study (IRS)-I, -II, and -III (1972–1989). Patients and Methods: A total of 171 children with primary RMS of the bladder were enrolled in the IRS during this interval. Partial cystectomy was performed in 40, in 33 before any other therapy and in seven after 10–57 weeks of chemotherapy (primarily vincristine, actinomycin-D, and cyclophosphamide, i.e., VAC) radiotherapy. Surviving patients have 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 two required bladder augmentation procedures for benign bladder contracture. Three quarters of the total group of living patients who have undergone partial cystectomy are without bladder-related symptoms or demonstrable lower urinary tract disease. The remaining patients have a history of functional bladder problems related to contracture or incontinence. Conclusions: Partial cystectomy is an alternative to total cystectomy for bladder RMS when the tumor site makes it anatomically feasible. 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.",
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T1 - Children with vesical rhabdomyosarcoma (RMS) treated by partial cystectomy with neoadjuvant or adjuvant chemotherapy, with or without radiotherapy

T2 - A report from the intergroup rhabdomyosarcoma study (IRS) committee

AU - Hays, Daniel M.

AU - Raney, R. Beverly

AU - Wharam, Moody D.

AU - Wiener, Eugene

AU - Lobe, Thom E.

AU - Andrassy, Richard J.

AU - Lawrence, Walter

AU - Johnston, Jean

AU - Webber, Bruce

AU - Maurer, Harold M.

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N2 - Purpose: Children with bladder rhabdomyosarcoma (RMS) are currently treated with primary chemotherapy and usually with local irradiation. More than 30% of this group ultimately require total cystectomy. The purpose 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 Intergroup Rhabdomyosarcoma Study (IRS)-I, -II, and -III (1972–1989). Patients and Methods: A total of 171 children with primary RMS of the bladder were enrolled in the IRS during this interval. Partial cystectomy was performed in 40, in 33 before any other therapy and in seven after 10–57 weeks of chemotherapy (primarily vincristine, actinomycin-D, and cyclophosphamide, i.e., VAC) radiotherapy. Surviving patients have 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 two required bladder augmentation procedures for benign bladder contracture. Three quarters of the total group of living patients who have undergone partial cystectomy are without bladder-related symptoms or demonstrable lower urinary tract disease. The remaining patients have a history of functional bladder problems related to contracture or incontinence. Conclusions: Partial cystectomy is an alternative to total cystectomy for bladder RMS when the tumor site makes it anatomically feasible. 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.

AB - Purpose: Children with bladder rhabdomyosarcoma (RMS) are currently treated with primary chemotherapy and usually with local irradiation. More than 30% of this group ultimately require total cystectomy. The purpose 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 Intergroup Rhabdomyosarcoma Study (IRS)-I, -II, and -III (1972–1989). Patients and Methods: A total of 171 children with primary RMS of the bladder were enrolled in the IRS during this interval. Partial cystectomy was performed in 40, in 33 before any other therapy and in seven after 10–57 weeks of chemotherapy (primarily vincristine, actinomycin-D, and cyclophosphamide, i.e., VAC) radiotherapy. Surviving patients have 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 two required bladder augmentation procedures for benign bladder contracture. Three quarters of the total group of living patients who have undergone partial cystectomy are without bladder-related symptoms or demonstrable lower urinary tract disease. The remaining patients have a history of functional bladder problems related to contracture or incontinence. Conclusions: Partial cystectomy is an alternative to total cystectomy for bladder RMS when the tumor site makes it anatomically feasible. 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.

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