Prognosis group¶ | Definition | Regimen | DoseΔ | Schedule |
Low risk | ||||
Subset A Excellent prognosis (>85% EFS) | Embryonal and alveolar fusion-negative tumors:
| 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:
| 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:
| 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:
| VAC × 14 cycles (40 weeks)‡[4] | ||
Vincristine | 1.5 mg/m2 (max 2 mg) | Weekly during weeks 1 to 13, 16, 19 to 25, 28, 31 to 37, and 40 | ||
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).CG: clinical group; COG: Children's Oncology Group; EFS: event-free survival; 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.Do you want to add Medilib to your home screen?