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Current standard chemotherapy regimens for newly diagnosed patients with rhabdomyosarcoma*

Current standard chemotherapy regimens for newly diagnosed patients with rhabdomyosarcoma*
Prognosis group Definition Regimen DoseΔ Schedule
Low risk

Subset A

Excellent prognosis

(>85% EFS)		 Embryonal and alveolar fusion-negative tumors:
  • Stage 1 CG I/II
  • Stage 1 CG III orbital
  • Stage 2 CG I/II
VA per subset A regimen of D9602 × 15 cycles (45 weeks)[1]
Vincristine 1.5 mg/m2 (max 2 mg) Weekly during weeks 0 to 8, 12 to 20, 24 to 32, and 36 to 44
Dactinomycin 0.045 mg/kg (max 2.5 mg) Every 3 weeks during weeks 0 through 45◊
OR
VAC/VA per subset A regimen of ARST0331 × 8 cycles (24 weeks)§[2]
Vincristine 1.5 mg/m2 (max 2 mg) Weekly during weeks 1 to 9 and 13 to 21
Dactinomycin 0.045 mg/kg (max 2.5 mg) Every 3 weeks during weeks 1 to 22◊
Cyclophosphamide 1200 mg/m2 with mesna and as-needed hematopoietic growth factor support Every 3 weeks during weeks 1 to 10 for a total of 4 doses

Subset B

Very good prognosis

(70 to 85% EFS)		 Embryonal and alveolar fusion-negative tumors:
  • Stage 1 CG III non-orbit
  • Stage 3 CG I/II
VAC × 14 cycles (40 weeks)¥[3]
Vincristine 1.5 mg/m2 (max 2 mg) Weekly during weeks 1 to 13, 16, 19 to 25, 28, 31, 34 to 37, and 40
Dactinomycin 0.045 mg/kg (max 2.5 mg) Every 3 weeks during weeks 1 through 40◊
Cyclophosphamide 2200 mg/m2 with mesna and hematopoietic growth factor support Every 3 weeks during weeks 1 through 40
Intermediate risk

Good prognosis

(50 to 70% EFS)		 Embryonal and alveolar fusion-negative tumors:
  • Stage 2/3 CG III
  • Metastatic disease, age <10 years
Alveolar fusion-positive tumors:
  • CG I to III
VAC × 14 cycles (40 weeks)¥[3]
Vincristine 1.5 mg/m2 (max 2 mg) Weekly during weeks 1 to 13, 16, 19 to 25, 28, 31, 34 to 37, and 40
Dactinomycin 0.045 mg/kg (max 2.5 mg) Every 3 weeks during weeks 1 through 40◊
Cyclophosphamide 2200 mg/m2 with mesna and hematopoietic growth factor support Every 3 weeks during weeks 1 through 40
High risk

Poor prognosis

(<30% EFS)		 Embryonal and alveolar fusion-negative tumors:
  • Metastatic disease, age >10 years
Alveolar fusion-positive tumors:
  • Metastatic disease, any age
VAC × 14 cycles (40 weeks)‡[4]
Vincristine 1.5 mg/m2 (max 2 mg) Weekly during weeks 1 to 13, 19 to 25, and 31 to 37
Dactinomycin 0.045 mg/kg (max 2.5 mg) Every 3 weeks during weeks 1 through 40◊
Cyclophosphamide 1200 mg/m2 with mesna and hematopoietic growth factor support Every 3 weeks during weeks 1 through 40
A pediatric oncologist with expertise in the soft tissue sarcoma field should be contacted for current standard therapy schemas.
The risk group descriptions in this table are based on the results of historically completed trials using the EFS estimates of the individual patient groups. Current COG protocols can deviate from theses definitions for protocol purposes (refer to text).
EFS: event-free survival; CG: clinical group; COG: Children's Oncology Group; RT: radiation therapy.
* RT, when indicated, is started at week 13 of chemotherapy as standard of care.
¶ Refer to the UpToDate table on rhabdomyosarcoma prognostic stratification for prognosis group definitions.
Δ Doses are for adults and children over the age of 3. Dose reductions are necessary for younger children.
◊ Omit dactinomycin on weeks 16 and 19 for patients beginning RT at week 12, weeks 17 and 21 for patients beginning RT at week 13, and weeks 31 and 34 for patients beginning RT at week 28.
§ This regimen is preferred for stage 1 CG IIb/c and stage 2 CG II.
¥ VAC doses and schedule are the same as were used in the COG study 9803.
‡ VAC doses and schedule are from the most recent COG protocol for intermediate-risk disease: ARST0531.
References:
  1. Raney RB, Walterhouse DO, Meza JL, et al. Results of the Intergroup Rhabdomyosarcoma Study Group D9602 protocol, using vincristine and dactinomycin with or without cyclophosphamide and radiation therapy, for newly diagnosed patients with low-risk embryonal rhabdomyosarcoma: a report from the Soft Tissue Sarcoma Committee of the Children's Oncology Group. J Clin Oncol 2011; 29:1312.
  2. Walterhouse DO, Pappo AS, Meza JL, et al. Reduction of cyclophosphamide dose for patients with subset 2 low-risk rhabdomyosarcoma is associated with an increased risk of recurrence: A report from the Soft Tissue Sarcoma Committee of the Children's Oncology Group. Cancer 2017; 123:2368.
  3. Arndt CA, Stoner JA, Hawkins DS, et al. 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; 27:5182.
  4. Hawkins DS, Chi YY, Anderson JR, et al. 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; 36:2770.
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