Rhabdomyosarcoma

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Section editor
Jim chen.jpeg
 James L. Chen, MD, MS
Columbus, OH
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4 regimens on this page
4 variants on this page


All lines of therapy

VAC

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VAC: Vincristine, Actinomycin D, Cyclophosphamide

Regimen

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

VAI

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VAI: Vincristine, Actinomycin D, Ifosfamide
IVA: Ifosfamide, Vincristine, Actinomycin D

Regimen

Study Evidence Comparator Efficacy
Crist et al. 2001 (IRS-IV) Phase III (E) VAC Seems not superior
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 it to HemOnc.org. Please refer to the original article.

References

  1. 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 Efficacy
Crist et al. 2001 (IRS-IV) Phase III (E) VAC Seems not superior
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
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