COG AREN0321, AREN0532, and AREN0533 |
Stage* | Histology and clinical factors | Chemotherapy | Radiation therapy |
I | Favorable histology; very low risk¶ | None | None |
Favorable histology; non-very low risk¶; without high-risk molecular markersΔ | Vincristine and dactinomycin for 19 weeks | None |
Favorable histology; non-very low risk¶; with high-risk molecular markersΔ | Vincristine, dactinomycin, and doxorubicin for 25 weeks | None |
Focal or diffuse anaplasia | Vincristine, dactinomycin, and doxorubicin for 25 weeks | 10.8 Gy flank |
II | Favorable histology; without high-risk molecular markersΔ | Vincristine and dactinomycin for 19 weeks | None |
Favorable histology; with high-risk molecular markersΔ | Vincristine, dactinomycin, and doxorubicin for 25 weeks | None |
Focal anaplasia | Vincristine, dactinomycin, and doxorubicin for 25 weeks | 10.8 Gy flank |
Diffuse anaplasia | Vincristine, doxorubicin, carboplatin, cyclophosphamide, and etoposide for 30 weeks | 10.8 Gy flank |
III | Favorable histology; without high-risk molecular markersΔ | Vincristine, dactinomycin, and doxorubicin for 25 weeks | 10.8 Gy flank/abdomen; 10.8 Gy boost for gross disease |
Favorable histology; with high-risk molecular markersΔ | Vincristine, dactinomycin, doxorubicin, cyclophosphamide, and etoposide for 31 weeks | 10.8 Gy flank/abdomen; 10.8 Gy boost for gross disease |
Focal anaplasia | Vincristine, dactinomycin, and doxorubicin for 25 weeks | 10.8 Gy flank/abdomen; 10.8 Gy boost for gross disease |
Diffuse anaplasia | Vincristine, doxorubicin, carboplatin, cyclophosphamide, and etoposide for 30 weeks | 20 Gy flank/abdomen; 10.8 Gy boost for gross disease |
IV | Favorable histology; without high-risk molecular markersΔ and lung nodule complete response at week 6 | Vincristine, dactinomycin, and doxorubicin for 25 weeks | No lung XRT; flank XRT for local stage III◊ |
Favorable histology; with high-risk molecular markersΔ and/or lung nodule incomplete response at week 6 | Vincristine, dactinomycin, doxorubicin, cyclophosphamide, and etoposide for 31 weeks | 12 Gy lung; flank XRT for local stage III◊ |
Focal anaplasia | Vincristine, doxorubicin, carboplatin, cyclophosphamide, and etoposide for 30 weeks | 12 Gy lung; flank XRT for local stage III◊ |
Diffuse anaplasia | Vincristine, doxorubicin, carboplatin, cyclophosphamide, etoposide, and irinotecan for 36 weeks | 12 Gy lung; flank XRT for local stage III◊ |