Rhabdomyosarcoma

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James L. Chen, MD, MS
The Ohio State University
Columbus, OH

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6 regimens on this page
6 variants on this page


All lines of therapy

Dactinomycin monotherapy

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Regimen

Study Evidence
Pinkel 1959 Pilot

This regimen is of historic interest.

Chemotherapy

References

  1. Pinkel D. Actinomycin D in childhood cancer; a preliminary report. Pediatrics. 1959 Feb;23(2):342-7. link to original article PubMed

VA

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VA: Vincristine & Actinomycin D

Regimen

Study Evidence Comparator Comparative Efficacy
Maurer et al. 1993 (IRS-II) Phase III (C) VAC Seems not superior
Crist et al. 1995 (IRS-III) Phase III (C) VAC Seems not superior

Note: reported efficacy is for group I favorable-histology tumors.

Chemotherapy

References

  1. IRS-II: Maurer HM, Gehan EA, Beltangady M, Crist W, Dickman PS, Donaldson SS, Fryer C, Hammond D, Hays DM, Herrmann J, Heyn R, Jones PM, Lawrence W, Newton W, Ortega J, Ragab AH, Raney RB, Ruymann FB, Soule E, Tefft M, Webber B, Wiener E, Wharam M, Vietti TJ. The Intergroup Rhabdomyosarcoma Study-II. Cancer. 1993 Mar 1;71(5):1904-22. link to original article PubMed
  2. IRS-III: Crist W, Gehan EA, Ragab AH, Dickman PS, Donaldson SS, Fryer C, Hammond D, Hays DM, Herrmann J, Heyn R, Jones PM, Lawrence W, Newton W, Ortega J, Raney RB, Ruymann FB, Tefft M, Webber B, Wiener E, Wharam M, Vietti TJ, Maurer HM. The Third Intergroup Rhabdomyosarcoma Study. J Clin Oncol. 1995 Mar;13(3):610-30. link to original article PubMed

VAC

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VAC: Vincristine, Actinomycin D, Cyclophosphamide

Regimen

Study Evidence Comparator Comparative Efficacy
Maurer et al. 1988 (IRS-I) Phase III (C) VAC & RT Seems not superior
Crist et al. 2001 (IRS-IV) Phase III (E) 1. VAI Seems not superior
2. VIE Seems not superior
Arndt et al. 2009 (COG D9803) Phase III (C) VAC/VTC Seems not superior
Hawkins et al. 2018 (ARST0531) Phase III (C) VAC/VI Seems not superior

To be completed

Chemotherapy

References

  1. IRS-I: Maurer HM, Beltangady M, Gehan EA, Crist W, Hammond D, Hays DM, Heyn R, Lawrence W, Newton W, Ortega J, Ragab AH, Raney RB, Ruymann FB, Soule E, Tefft M, Webber B, Wharam M, Vietti TJ. The Intergroup Rhabdomyosarcoma Study-I: a final report. Cancer. 1988 Jan 15;61(2):209-20. link to original article PubMed
  2. IRS-IV: Crist WM, Anderson JR, Meza JL, Fryer C, Raney RB, Ruymann FB, Breneman J, Qualman SJ, Wiener E, Wharam M, Lobe T, Webber B, Maurer HM, Donaldson SS. Intergroup Rhabdomyosarcoma Study-IV: results for patients with nonmetastatic disease. J Clin Oncol. 2001 Jun 15;19(12):3091-102. link to original article PubMed
  3. COG D9803: Arndt CA, Stoner JA, Hawkins DS, Rodeberg DA, Hayes-Jordan AA, Paidas CN, Parham DM, Teot LA, Wharam MD, Breneman JC, Donaldson SS, Anderson JR, Meyer WH. Vincristine, actinomycin, and cyclophosphamide compared with vincristine, actinomycin, and cyclophosphamide alternating with vincristine, topotecan, and cyclophosphamide for intermediate-risk rhabdomyosarcoma: Children's Oncology Group study D9803. J Clin Oncol. 2009 Nov 1;27(31):5182-8. Epub 2009 Sep 21. link to original article link to PMC article PubMed
  4. ARST0531: Hawkins DS, Chi YY, Anderson JR, Tian J, Arndt CAS, Bomgaars L, Donaldson SS, Hayes-Jordan A, Mascarenhas L, McCarville MB, McCune JS, McCowage G, Million L, Morris CD, Parham DM, Rodeberg DA, Rudzinski ER, Shnorhavorian M, Spunt SL, Skapek SX, Teot LA, Wolden S, Yock TI, Meyer WH. Addition of vincristine and irinotecan to vincristine, dactinomycin, and cyclophosphamide does not improve outcome for intermediate-risk rhabdomyosarcoma: a report from the Children's Oncology Group. J Clin Oncol. 2018 Sep 20;36(27):2770-2777. Epub 2018 Aug 9. link to original article link to PMC article PubMed

VAI

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VAI: Vincristine, Actinomycin-D (Dactinomycin), Ifosfamide
IVA: Ifosfamide, Vincristine, Actinomycin-D (Dactinomycin)

Regimen

Study Evidence Comparator Comparative Efficacy
Crist et al. 2001 (IRS-IV) Phase III (E) 1. VAC Seems not superior
2. VIE Seems not superior
Oberlin et al. 2012 (SIOP MMT95) Phase III (C) IVA/CEV/IVE Seems not superior
Bisogno et al. 2018 (EpSSG RMS 2005) Phase III (C) IVA-D, then IVA Seems not superior

To be completed

Chemotherapy

References

  1. IRS-IV: Crist WM, Anderson JR, Meza JL, Fryer C, Raney RB, Ruymann FB, Breneman J, Qualman SJ, Wiener E, Wharam M, Lobe T, Webber B, Maurer HM, Donaldson SS. Intergroup Rhabdomyosarcoma Study-IV: results for patients with nonmetastatic disease. J Clin Oncol. 2001 Jun 15;19(12):3091-102. link to original article PubMed
  2. SIOP MMT95: Oberlin O, Rey A, Sanchez de Toledo J, Martelli H, Jenney ME, Scopinaro M, Bergeron C, Merks JH, Bouvet N, Ellershaw C, Kelsey A, Spooner D, Stevens MC. Randomized comparison of intensified six-drug versus standard three-drug chemotherapy for high-risk nonmetastatic rhabdomyosarcoma and other chemotherapy-sensitive childhood soft tissue sarcomas: long-term results from the International Society of Pediatric Oncology MMT95 study. J Clin Oncol. 2012 Jul 10;30(20):2457-65. Epub 2012 Jun 4. link to original article PubMed
  3. EpSSG RMS 2005: Bisogno G, Jenney M, Bergeron C, Gallego Melcón S, Ferrari A, Oberlin O, Carli M, Stevens M, Kelsey A, De Paoli A, Gaze MN, Martelli H, Devalck C, Merks JH, Ben-Arush M, Glosli H, Chisholm J, Orbach D, Minard-Colin V, De Salvo GL; European paediatric Soft tissue sarcoma Study Group. Addition of dose-intensified doxorubicin to standard chemotherapy for rhabdomyosarcoma (EpSSG RMS 2005): a multicentre, open-label, randomised controlled, phase 3 trial. Lancet Oncol. 2018 Aug;19(8):1061-1071. Epub 2018 Jun 22. link to original article PubMed

VI, then VDC/IE, then VAC, then VI

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VI, then VDC/IE, then VAC, then VI: Vincristine & Irinotecan, followed by Vincristine, Doxorubicin, Cyclophosphamide alternating with Ifosfamide & Etoposide, followed by Vincristine, Actinomycin D, Cyclophosphamide, followed by Vincristine & Irinotecan

Regimen

Study Evidence
Weigel et al. 2015 (COG ARST0431) Phase II

Note: this is a complicated 54-week regimen with no immediate plans to add details to HemOnc.org. Please refer to the original article.

Chemotherapy, VI portion

Chemotherapy, VDC portion

Chemotherapy, IE portion

Chemotherapy, VAC portion

References

  1. COG ARST0431: Weigel BJ, Lyden E, Anderson JR, Meyer WH, Parham DM, Rodeberg DA, Michalski JM, Hawkins DS, Arndt CA. Intensive multiagent therapy, including dose-compressed cycles of ifosfamide/etoposide and vincristine/doxorubicin/cyclophosphamide, irinotecan, and radiation, in patients with high-risk rhabdomyosarcoma: A report from the Children's Oncology Group. J Clin Oncol. 2016 Jan 10;34(2):117-22. Epub 2015 Oct 26. link to original article link to PMC article PubMed

VIE

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VIE: Vincristine, Ifosfamide, Etoposide

Regimen

Study Evidence Comparator Comparative Efficacy
Crist et al. 2001 (IRS-IV) Phase III (E) 1. VAC Seems not superior
2. VAI Seems not superior

To be completed

Chemotherapy

References

  1. IRS-IV: Crist WM, Anderson JR, Meza JL, Fryer C, Raney RB, Ruymann FB, Breneman J, Qualman SJ, Wiener E, Wharam M, Lobe T, Webber B, Maurer HM, Donaldson SS. Intergroup Rhabdomyosarcoma Study-IV: results for patients with nonmetastatic disease. J Clin Oncol. 2001 Jun 15;19(12):3091-102. link to original article PubMed