The intergroup rhabdomyosarcoma study. A preliminary report

Harold M. Maurer, Thomas Moon, Milton Donaldson, Carlos Fernandez, Edmund A. Gehan, Denman Hammond, Daniel M. Hays, Walter Lawrence, William Newton, Abdelsalam Ragab, Beverly Raney, Edward H. Soule, W. W. Sutow, Melvin Tefft

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282 Scopus citations

Abstract

Four hundred and twenty‐three children with newly diagnosed rhabdomyosarcoma have been entered to date in the Intergroup Rhabdomyosarcoma Study (IRS), which began in 1972. Patients were classified into Clinical Disease Groups (Stages) I‐IV, based on disease extent and resectability, and treatment regimens were randomly assigned according to group. Two hundred and seventy‐eight of 423 patients are evaluable for this analysis. Thus far, for Group I disease (localized/completely resected), disease control achieved by vincristine, dactinomycin, and cyclophosphamide (VAC) given in combination for 2 years has not been enhanced by the administration of postoperative radiation to the tumor bed. To date, 92% of patients in both irradiated and nonirradiated groups exhibit no evidence of disease, and 92‐96% are still alive, with the median time of follow‐up being 72 weeks. For Group II disease (microscopic residual/nodal involvement), VAC given for 2 years has not been found to be more effective than vincristine plus dactinomycin given for 1 year, both groups also having received postoperative irradiation. Thus far, over 85% of patients on either treatment have no evidence of disease and 90% are still alive. Survival and complete remission durations range from 1+ to 143+ weeks and the median duration of follow‐up is 45 weeks. Chemotherapy as initial treatment has been studied in Group III (localized sarcoma not treated initially by gross total resection) and Group IV (metastases present at diagnosis) patients. They have received either intensive “pulse” VAC or “pulse” VAC plus Adriamycin, and radiation has been administered after 6 weeks. Eighty‐one percent of patients in Group III and 81‐83% in Group IV have responded favorably, with complete regression of disease having been observed in over one‐fourth of patients even before the start of radiation and in approximately one‐half of all the patients after receiving radiation therapy. There is no indication as yet that one treatment regimen is superior to the other. Seventy‐nine percent of patients in Group III are still alive (0+ to 154+ weeks) and 69% remain in continuous response (0+ to 139+ weeks) with the median duration of follow‐up being 41‐44 weeks. Fifty percent of patients in Group IV are still alive (0+ to 127+ weeks) with a median time of follow‐up of 41‐44 weeks. Tumors arising either from genitourinary sites or the extremities have had a higher incidence of lymphatic spread than tumors in all other primary sites of origin.

Original languageEnglish (US)
Pages (from-to)2015-2026
Number of pages12
JournalCancer
Volume40
Issue number5
DOIs
StatePublished - Nov 1977

ASJC Scopus subject areas

  • Oncology
  • Cancer Research

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    Maurer, H. M., Moon, T., Donaldson, M., Fernandez, C., Gehan, E. A., Hammond, D., Hays, D. M., Lawrence, W., Newton, W., Ragab, A., Raney, B., Soule, E. H., Sutow, W. W., & Tefft, M. (1977). The intergroup rhabdomyosarcoma study. A preliminary report. Cancer, 40(5), 2015-2026. https://doi.org/10.1002/1097-0142(197711)40:5<2015::AID-CNCR2820400505>3.0.CO;2-K