Rhabdomyosarcoma

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Section editor
Elizabethdavis2.jpg
 Elizabeth J. Davis, MD
Vanderbilt University
Nashville, TN, USA

Note: these are regimens tested in subtype-specific populations, please see the main soft tissue sarcoma page for other regimens. Pleomorphic rhabdomyosarcoma is typically treated the same as soft tissue sarcoma.
Note: certain regimens are to be found on dedicated pages:

5 regimens on this page
5 variants on this page


Guidelines

Given the rapid change in evidence in many areas of hematology/oncology, readers are encouraged to consider any guideline published 5+ years ago to be for historical purposes, only.

NCCN

All lines of therapy

VA

VA: Vincristine & Actinomycin D

Regimen

Study Dates of enrollment Evidence Comparator Comparative Efficacy
Maurer et al. 1993 (IRS-II) 1978-1984 Phase 3 (C) VAC Did not meet co-primary endpoints of DFS/OS
Crist et al. 1995 (IRS-III) 1984-1991 Phase 3 (C) VAC Did not meet primary endpoint of PFS

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

VAC: Vincristine, Actinomycin D, Cyclophosphamide

Regimen

Study Dates of enrollment Evidence Comparator Comparative Efficacy
Maurer et al. 1988 (IRS-I) 1972-1978 Phase 3 (C) VAC & RT Did not meet co-primary endpoints of DFS/OS
Crist et al. 1995 (IRS-III) 1984-1991 Phase 3 (E-RT-esc) VA Did not meet primary endpoint of PFS
Crist et al. 2001 (IRS-IV) 1991-1997 Phase 3 (E-switch-ic) 1. VAI Did not meet co-primary endpoints of FFS/OS
2. VIE Did not meet co-primary endpoints of FFS/OS
Arndt et al. 2009 (COG D9803) 1999-2005 Phase 3 (C) VAC/VTC Did not meet primary endpoint of FFS
Hawkins et al. 2018 (COG ARST0531) 2006-2012 Phase 3 (C) VAC/VI Did not meet primary endpoint of EFS

Note: The first week of this protocol is week 0.

Chemotherapy

104-week course

References

  1. IRS-I: Maurer HM, Moon T, Donaldson M, Fernandez C, Gehan EA, Hammond D, Hays DM, Lawrence W Jr, Newton W, Ragab A, Raney B, Soule EH, Sutow WW, Tefft M. The intergroup rhabdomyosarcoma study: a preliminary report. Cancer. 1977 Nov;40(5):2015-26. link to original article contains dosing details in manuscript PubMed
    1. Update: 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-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
  3. 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
  4. 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 NCT00003958
  5. COG 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; Children's Oncology Group. 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 NCT00354835

VAI

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

Regimen

Study Dates of enrollment Evidence Comparator Comparative Efficacy
Crist et al. 2001 (IRS-IV) 1991-1997 Phase 3 (E-switch-ic) 1. VAC Did not meet co-primary endpoints of FFS/OS
2. VIE Did not meet co-primary endpoints of FFS/OS
Oberlin et al. 2012 (SIOP MMT95) 1995-2003 Phase 3 (C) IVA/CEV/IVE Did not meet primary endpoint of OS
Bisogno et al. 2018 (EpSSG RMS 2005) 2006-2016 Phase 3 (C) IVA-D, then IVA Did not meet primary endpoint of EFS36

Chemotherapy

Supportive therapy

21-day cycle for 3 cycles

Subsequent treatment

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 contains dosing details in manuscript 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 NCT00002898
  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 NCT00339118
    1. Update: Bisogno G, De Salvo GL, Bergeron C, Gallego Melcón S, Merks JH, Kelsey A, Martelli H, Minard-Colin V, Orbach D, Glosli H, Chisholm J, Casanova M, Zanetti I, Devalck C, Ben-Arush M, Mudry P, Ferman S, Jenney M, Ferrari A; European paediatric Soft tissue sarcoma Study Group. Vinorelbine and continuous low-dose cyclophosphamide as maintenance chemotherapy in patients with high-risk rhabdomyosarcoma (RMS 2005): a multicentre, open-label, randomised, phase 3 trial. Lancet Oncol. 2019 Nov;20(11):1566-1575. Epub 2019 Sep 24. link to original article PubMed
    2. Update: Bisogno G, Minard-Colin V, Zanetti I, Ferrari A, Gallego S, Dávila Fajardo R, Mandeville H, Kelsey A, Alaggio R, Orbach D, Terwisscha van Scheltinga S, Guillén Burrieza G, Ben-Arush M, Glosli H, Mudry P, Ferman S, Devalck C, Defachelles AS, Merks JHM, Jenney M. Nonmetastatic Rhabdomyosarcoma in Children and Adolescents: Overall Results of the European Pediatric Soft Tissue Sarcoma Study Group RMS2005 Study. J Clin Oncol. 2023 May 1;41(13):2342-2349. Epub 2023 Feb 27. link to original article PubMed

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

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 Dates of enrollment Evidence
Weigel et al. 2015 (COG ARST0431) 2006-07-17 to 2008-06-13 Non-randomized

Note: this is a complicated 54-week protocol 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 NCT00354744

VIE

VIE: Vincristine, Ifosfamide, Etoposide

Regimen

Study Dates of enrollment Evidence Comparator Comparative Efficacy
Crist et al. 2001 (IRS-IV) 1991-1997 Phase 3 (E-switch-ic) 1. VAC Did not meet co-primary endpoints of FFS/OS
2. VAI Did not meet co-primary endpoints of FFS/OS

Chemotherapy

Supportive therapy

21-day cycle for 3 cycles

Subsequent treatment

  • VI & RT consolidation, then VIE consolidation x 1, then VIE continuation

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 contains dosing details in manuscript PubMed
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