Partial cystectomy in the management of rhabdomyosarcoma of the bladder

A report from the intergroup rhabdomyosarcoma study

D. M. Hays, W. Lawrence, W. M. Crist, E. Wiener, R. B. Raney, A. Ragab, M. Teffr, B. Webber, J. Johnston, Harold Maurice Maurer, Rhabdomyosarcoma Study Intergroup Rhabdomyosarcoma Study

Research output: Contribution to journalArticle

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Abstract

Among 154 children with primary vesical rhabdomyosarcoma entered in the IRS (1972 to 1986), tumor excision constituting a partial cystectomy was performed in 33. These procedures were performed in the following situations (1) as an initial operation for localized disease (22): (2) as a secondary procedure for localized disease following chemotherapy (CT) or CT/irradiation response (6); and (3) as an initial operation in the presence of disseminated abdominal disease (5). Partial cystectomy included fullthickness resection of 15% to 80% of the bladder wall. Ureters were reimplanted or revised in two patients. The 33 patients undergoing partial cystectomy were in the following clinical groups: I, total excision (10); II, gross excision with "microscopic residual" or positive nodes (8); III, subtotal excision (10); and IV, tumor dissemination (5). Patients in clinical groups I and II received vincristine and actinomycin-D (VA) or VA + cyclophosphamide (VAC) ± adriamycin (ADR). Patients in groups III and IV received the same agents ± cisplatin. Irradiation (2,000 to 5,000 cGy) was administered to 18 patients (55%) in groups II to IV. Six relapses occurred among 28 patients with localized disease, and one among five patients with dissemination; all resulting in progressive disease and/or death. Estimated 3-year survival following partial cystectomy (79%) is similar to that for all patients with primary bladder tumors (78%). In contrast with the total group, in which the majority of survivors have eventually required total cystectomy; 25 of 26 survivors of partial cystectomy have functional bladders from 146 to 686 weeks (median 356 weeks) from study entry. Partial cystectomy should be considered as an option for patients with rhabdomyosarcoma of the bladder before either performing total cystectomy or relying solely on chemotherapy/radiotherapy regimens.

Original languageEnglish (US)
Pages (from-to)719-723
Number of pages5
JournalJournal of pediatric surgery
Volume25
Issue number7
DOIs
StatePublished - Jan 1 1990

Fingerprint

Rhabdomyosarcoma
Cystectomy
Urinary Bladder
Dactinomycin
Vincristine
Drug Therapy
Survivors
Ureter
Urinary Bladder Neoplasms
Doxorubicin
Cyclophosphamide
Cisplatin
Neoplasms
Radiotherapy
Recurrence
Survival

Keywords

  • Vesical rhabdomyosarcoma
  • partial cystectomy

ASJC Scopus subject areas

  • Surgery
  • Pediatrics, Perinatology, and Child Health

Cite this

Hays, D. M., Lawrence, W., Crist, W. M., Wiener, E., Raney, R. B., Ragab, A., ... Intergroup Rhabdomyosarcoma Study, R. S. (1990). Partial cystectomy in the management of rhabdomyosarcoma of the bladder: A report from the intergroup rhabdomyosarcoma study. Journal of pediatric surgery, 25(7), 719-723. https://doi.org/10.1016/S0022-3468(05)80004-1

Partial cystectomy in the management of rhabdomyosarcoma of the bladder : A report from the intergroup rhabdomyosarcoma study. / Hays, D. M.; Lawrence, W.; Crist, W. M.; Wiener, E.; Raney, R. B.; Ragab, A.; Teffr, M.; Webber, B.; Johnston, J.; Maurer, Harold Maurice; Intergroup Rhabdomyosarcoma Study, Rhabdomyosarcoma Study.

In: Journal of pediatric surgery, Vol. 25, No. 7, 01.01.1990, p. 719-723.

Research output: Contribution to journalArticle

Hays, DM, Lawrence, W, Crist, WM, Wiener, E, Raney, RB, Ragab, A, Teffr, M, Webber, B, Johnston, J, Maurer, HM & Intergroup Rhabdomyosarcoma Study, RS 1990, 'Partial cystectomy in the management of rhabdomyosarcoma of the bladder: A report from the intergroup rhabdomyosarcoma study', Journal of pediatric surgery, vol. 25, no. 7, pp. 719-723. https://doi.org/10.1016/S0022-3468(05)80004-1
Hays, D. M. ; Lawrence, W. ; Crist, W. M. ; Wiener, E. ; Raney, R. B. ; Ragab, A. ; Teffr, M. ; Webber, B. ; Johnston, J. ; Maurer, Harold Maurice ; Intergroup Rhabdomyosarcoma Study, Rhabdomyosarcoma Study. / Partial cystectomy in the management of rhabdomyosarcoma of the bladder : A report from the intergroup rhabdomyosarcoma study. In: Journal of pediatric surgery. 1990 ; Vol. 25, No. 7. pp. 719-723.
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abstract = "Among 154 children with primary vesical rhabdomyosarcoma entered in the IRS (1972 to 1986), tumor excision constituting a partial cystectomy was performed in 33. These procedures were performed in the following situations (1) as an initial operation for localized disease (22): (2) as a secondary procedure for localized disease following chemotherapy (CT) or CT/irradiation response (6); and (3) as an initial operation in the presence of disseminated abdominal disease (5). Partial cystectomy included fullthickness resection of 15{\%} to 80{\%} of the bladder wall. Ureters were reimplanted or revised in two patients. The 33 patients undergoing partial cystectomy were in the following clinical groups: I, total excision (10); II, gross excision with {"}microscopic residual{"} or positive nodes (8); III, subtotal excision (10); and IV, tumor dissemination (5). Patients in clinical groups I and II received vincristine and actinomycin-D (VA) or VA + cyclophosphamide (VAC) ± adriamycin (ADR). Patients in groups III and IV received the same agents ± cisplatin. Irradiation (2,000 to 5,000 cGy) was administered to 18 patients (55{\%}) in groups II to IV. Six relapses occurred among 28 patients with localized disease, and one among five patients with dissemination; all resulting in progressive disease and/or death. Estimated 3-year survival following partial cystectomy (79{\%}) is similar to that for all patients with primary bladder tumors (78{\%}). In contrast with the total group, in which the majority of survivors have eventually required total cystectomy; 25 of 26 survivors of partial cystectomy have functional bladders from 146 to 686 weeks (median 356 weeks) from study entry. Partial cystectomy should be considered as an option for patients with rhabdomyosarcoma of the bladder before either performing total cystectomy or relying solely on chemotherapy/radiotherapy regimens.",
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