The intergroup rhabdomyosarcoma study‐I. A final report

Harold M. Maurer, William Crist, Walter Lawrence, Abdelsalam H. Ragab, R. Beverly Raney, Bruce Webber, Moody Wharam, Teresa J. Vietti, Mohan Beltangady, Edmund A. Gehan, Denman Hammond, Daniel M. Hays, Ruth Heyn, William Newton, Jorge Ortega, Frederick B. Ruymann, Edward Soule, Melvin Tefft

Research output: Contribution to journalArticle

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Abstract

The results of treatment of 686, previously untreated patients younger than 21 years with rhabdomyosarcoma or undifferentiated sarcoma, who were entered on Intergroup Rhabdomyosarcoma Study‐I (IRS‐I) were analyzed after a minimum potential follow‐up time of 7 years. Patients in Clinical Group I (localized disease, completely resected) were randomized to receive either vincristine, dactinomycin, and cyclophosphamide (VAC) or VAC + radiation. At 5 years, approximately 80% of patients given either treatment were still disease‐free and there was no significant difference between treatments in the overall percentages of patients surviving of 93% and 81%, respectively (P = 0.67). Patients in Clinical Group II (regional disease, grossly resected) were randomized to receive either vincristine and dactinomycin (VA) + radiation or VAC + radiation. At 5 years, 72% and 65% of the patients, respectively, were disease‐free and there was no evidence of a difference between treatments (P = 0.46). The overall survival percentage at 5 years was approximately 72% for both treatments. Patients in Clinical Groups III (gross residual disease after surgery) and IV (metastatic disease) were randomized to receive either “pulse” VAC + radiation or “pulse” VAC + Adriamycin (doxorubicin) + radiation. The complete remission (CR) rate was 69% in Clinical Group III and 50% in IV, with no statistically significant difference in CR rates between treatments in either group. Those who achieved a CR had a nearly 60% chance of staying in remission for 5 years in Clinical Group III compared with approximately 30% in Clinical Group IV. The overall survival percentage at 5 years was 52% in Clinical Group III compared to 20% in Clinical Group IV (P < 0.0001). The 5‐year survival percentage for the entire cohort of 686 patients was 55%. Survival after relapse was poor, being 32% at 1 year and 17% at 2 years. The risk of distant metastasis was much greater than the risk of local recurrence within each clinical group, and there was no evidence of differing types of relapses between treatments. Primary tumors of the orbit and genitourinary tract carried the best prognosis, whereas tumors of the retroperitoneum had the worst prognosis. The authors conclude that for the therapeutic regimens evaluated there was no therapeutic advantage to including radiation in the treatment of Clinical Group I disease, or cyclophosphamide given as a daily low‐dose oral regimen in the treatment of Clinical Group II disease or Adriamycin in the treatment of Clinical Groups III and IV diseases.

Original languageEnglish (US)
Pages (from-to)209-220
Number of pages12
JournalCancer
Volume61
Issue number2
DOIs
StatePublished - Jan 15 1988

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Rhabdomyosarcoma
Dactinomycin
Vincristine
Cyclophosphamide
Radiation
Therapeutics
Doxorubicin
Survival
Recurrence
Orbit
Sarcoma
Neoplasms

ASJC Scopus subject areas

  • Oncology
  • Cancer Research

Cite this

Maurer, H. M., Crist, W., Lawrence, W., Ragab, A. H., Raney, R. B., Webber, B., ... Tefft, M. (1988). The intergroup rhabdomyosarcoma study‐I. A final report. Cancer, 61(2), 209-220. https://doi.org/10.1002/1097-0142(19880115)61:2<209::AID-CNCR2820610202>3.0.CO;2-L

The intergroup rhabdomyosarcoma study‐I. A final report. / Maurer, Harold M.; Crist, William; Lawrence, Walter; Ragab, Abdelsalam H.; Raney, R. Beverly; Webber, Bruce; Wharam, Moody; Vietti, Teresa J.; Beltangady, Mohan; Gehan, Edmund A.; Hammond, Denman; Hays, Daniel M.; Heyn, Ruth; Newton, William; Ortega, Jorge; Ruymann, Frederick B.; Soule, Edward; Tefft, Melvin.

In: Cancer, Vol. 61, No. 2, 15.01.1988, p. 209-220.

Research output: Contribution to journalArticle

Maurer, HM, Crist, W, Lawrence, W, Ragab, AH, Raney, RB, Webber, B, Wharam, M, Vietti, TJ, Beltangady, M, Gehan, EA, Hammond, D, Hays, DM, Heyn, R, Newton, W, Ortega, J, Ruymann, FB, Soule, E & Tefft, M 1988, 'The intergroup rhabdomyosarcoma study‐I. A final report', Cancer, vol. 61, no. 2, pp. 209-220. https://doi.org/10.1002/1097-0142(19880115)61:2<209::AID-CNCR2820610202>3.0.CO;2-L
Maurer HM, Crist W, Lawrence W, Ragab AH, Raney RB, Webber B et al. The intergroup rhabdomyosarcoma study‐I. A final report. Cancer. 1988 Jan 15;61(2):209-220. https://doi.org/10.1002/1097-0142(19880115)61:2<209::AID-CNCR2820610202>3.0.CO;2-L
Maurer, Harold M. ; Crist, William ; Lawrence, Walter ; Ragab, Abdelsalam H. ; Raney, R. Beverly ; Webber, Bruce ; Wharam, Moody ; Vietti, Teresa J. ; Beltangady, Mohan ; Gehan, Edmund A. ; Hammond, Denman ; Hays, Daniel M. ; Heyn, Ruth ; Newton, William ; Ortega, Jorge ; Ruymann, Frederick B. ; Soule, Edward ; Tefft, Melvin. / The intergroup rhabdomyosarcoma study‐I. A final report. In: Cancer. 1988 ; Vol. 61, No. 2. pp. 209-220.
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abstract = "The results of treatment of 686, previously untreated patients younger than 21 years with rhabdomyosarcoma or undifferentiated sarcoma, who were entered on Intergroup Rhabdomyosarcoma Study‐I (IRS‐I) were analyzed after a minimum potential follow‐up time of 7 years. Patients in Clinical Group I (localized disease, completely resected) were randomized to receive either vincristine, dactinomycin, and cyclophosphamide (VAC) or VAC + radiation. At 5 years, approximately 80{\%} of patients given either treatment were still disease‐free and there was no significant difference between treatments in the overall percentages of patients surviving of 93{\%} and 81{\%}, respectively (P = 0.67). Patients in Clinical Group II (regional disease, grossly resected) were randomized to receive either vincristine and dactinomycin (VA) + radiation or VAC + radiation. At 5 years, 72{\%} and 65{\%} of the patients, respectively, were disease‐free and there was no evidence of a difference between treatments (P = 0.46). The overall survival percentage at 5 years was approximately 72{\%} for both treatments. Patients in Clinical Groups III (gross residual disease after surgery) and IV (metastatic disease) were randomized to receive either “pulse” VAC + radiation or “pulse” VAC + Adriamycin (doxorubicin) + radiation. The complete remission (CR) rate was 69{\%} in Clinical Group III and 50{\%} in IV, with no statistically significant difference in CR rates between treatments in either group. Those who achieved a CR had a nearly 60{\%} chance of staying in remission for 5 years in Clinical Group III compared with approximately 30{\%} in Clinical Group IV. The overall survival percentage at 5 years was 52{\%} in Clinical Group III compared to 20{\%} in Clinical Group IV (P < 0.0001). The 5‐year survival percentage for the entire cohort of 686 patients was 55{\%}. Survival after relapse was poor, being 32{\%} at 1 year and 17{\%} at 2 years. The risk of distant metastasis was much greater than the risk of local recurrence within each clinical group, and there was no evidence of differing types of relapses between treatments. Primary tumors of the orbit and genitourinary tract carried the best prognosis, whereas tumors of the retroperitoneum had the worst prognosis. The authors conclude that for the therapeutic regimens evaluated there was no therapeutic advantage to including radiation in the treatment of Clinical Group I disease, or cyclophosphamide given as a daily low‐dose oral regimen in the treatment of Clinical Group II disease or Adriamycin in the treatment of Clinical Groups III and IV diseases.",
author = "Maurer, {Harold M.} and William Crist and Walter Lawrence and Ragab, {Abdelsalam H.} and Raney, {R. Beverly} and Bruce Webber and Moody Wharam and Vietti, {Teresa J.} and Mohan Beltangady and Gehan, {Edmund A.} and Denman Hammond and Hays, {Daniel M.} and Ruth Heyn and William Newton and Jorge Ortega and Ruymann, {Frederick B.} and Edward Soule and Melvin Tefft",
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T1 - The intergroup rhabdomyosarcoma study‐I. A final report

AU - Maurer, Harold M.

AU - Crist, William

AU - Lawrence, Walter

AU - Ragab, Abdelsalam H.

AU - Raney, R. Beverly

AU - Webber, Bruce

AU - Wharam, Moody

AU - Vietti, Teresa J.

AU - Beltangady, Mohan

AU - Gehan, Edmund A.

AU - Hammond, Denman

AU - Hays, Daniel M.

AU - Heyn, Ruth

AU - Newton, William

AU - Ortega, Jorge

AU - Ruymann, Frederick B.

AU - Soule, Edward

AU - Tefft, Melvin

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N2 - The results of treatment of 686, previously untreated patients younger than 21 years with rhabdomyosarcoma or undifferentiated sarcoma, who were entered on Intergroup Rhabdomyosarcoma Study‐I (IRS‐I) were analyzed after a minimum potential follow‐up time of 7 years. Patients in Clinical Group I (localized disease, completely resected) were randomized to receive either vincristine, dactinomycin, and cyclophosphamide (VAC) or VAC + radiation. At 5 years, approximately 80% of patients given either treatment were still disease‐free and there was no significant difference between treatments in the overall percentages of patients surviving of 93% and 81%, respectively (P = 0.67). Patients in Clinical Group II (regional disease, grossly resected) were randomized to receive either vincristine and dactinomycin (VA) + radiation or VAC + radiation. At 5 years, 72% and 65% of the patients, respectively, were disease‐free and there was no evidence of a difference between treatments (P = 0.46). The overall survival percentage at 5 years was approximately 72% for both treatments. Patients in Clinical Groups III (gross residual disease after surgery) and IV (metastatic disease) were randomized to receive either “pulse” VAC + radiation or “pulse” VAC + Adriamycin (doxorubicin) + radiation. The complete remission (CR) rate was 69% in Clinical Group III and 50% in IV, with no statistically significant difference in CR rates between treatments in either group. Those who achieved a CR had a nearly 60% chance of staying in remission for 5 years in Clinical Group III compared with approximately 30% in Clinical Group IV. The overall survival percentage at 5 years was 52% in Clinical Group III compared to 20% in Clinical Group IV (P < 0.0001). The 5‐year survival percentage for the entire cohort of 686 patients was 55%. Survival after relapse was poor, being 32% at 1 year and 17% at 2 years. The risk of distant metastasis was much greater than the risk of local recurrence within each clinical group, and there was no evidence of differing types of relapses between treatments. Primary tumors of the orbit and genitourinary tract carried the best prognosis, whereas tumors of the retroperitoneum had the worst prognosis. The authors conclude that for the therapeutic regimens evaluated there was no therapeutic advantage to including radiation in the treatment of Clinical Group I disease, or cyclophosphamide given as a daily low‐dose oral regimen in the treatment of Clinical Group II disease or Adriamycin in the treatment of Clinical Groups III and IV diseases.

AB - The results of treatment of 686, previously untreated patients younger than 21 years with rhabdomyosarcoma or undifferentiated sarcoma, who were entered on Intergroup Rhabdomyosarcoma Study‐I (IRS‐I) were analyzed after a minimum potential follow‐up time of 7 years. Patients in Clinical Group I (localized disease, completely resected) were randomized to receive either vincristine, dactinomycin, and cyclophosphamide (VAC) or VAC + radiation. At 5 years, approximately 80% of patients given either treatment were still disease‐free and there was no significant difference between treatments in the overall percentages of patients surviving of 93% and 81%, respectively (P = 0.67). Patients in Clinical Group II (regional disease, grossly resected) were randomized to receive either vincristine and dactinomycin (VA) + radiation or VAC + radiation. At 5 years, 72% and 65% of the patients, respectively, were disease‐free and there was no evidence of a difference between treatments (P = 0.46). The overall survival percentage at 5 years was approximately 72% for both treatments. Patients in Clinical Groups III (gross residual disease after surgery) and IV (metastatic disease) were randomized to receive either “pulse” VAC + radiation or “pulse” VAC + Adriamycin (doxorubicin) + radiation. The complete remission (CR) rate was 69% in Clinical Group III and 50% in IV, with no statistically significant difference in CR rates between treatments in either group. Those who achieved a CR had a nearly 60% chance of staying in remission for 5 years in Clinical Group III compared with approximately 30% in Clinical Group IV. The overall survival percentage at 5 years was 52% in Clinical Group III compared to 20% in Clinical Group IV (P < 0.0001). The 5‐year survival percentage for the entire cohort of 686 patients was 55%. Survival after relapse was poor, being 32% at 1 year and 17% at 2 years. The risk of distant metastasis was much greater than the risk of local recurrence within each clinical group, and there was no evidence of differing types of relapses between treatments. Primary tumors of the orbit and genitourinary tract carried the best prognosis, whereas tumors of the retroperitoneum had the worst prognosis. The authors conclude that for the therapeutic regimens evaluated there was no therapeutic advantage to including radiation in the treatment of Clinical Group I disease, or cyclophosphamide given as a daily low‐dose oral regimen in the treatment of Clinical Group II disease or Adriamycin in the treatment of Clinical Groups III and IV diseases.

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