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. 2015 Aug 24;33(27):2999–3007. doi: 10.1200/JCO.2015.62.1888

Table 2.

SIOP-2001 Treatment Approach

Stage Preoperative Chemotherapy Histology Other Clinical or Biologic Factor Postoperative Chemotherapy XRT
I AV × 4 weeks Low risk None None
Intermediate risk Postoperative tumor volume > 500 mL* AV × 4 weeks None
High risk AVD × 27 weeks None
II AV × 4 weeks Low risk AV × 27 weeks None
Intermediate risk Postoperative tumor volume > 500 mL* AV × 27 weeks v AVD × 27 weeks None
High risk DCBE × 34 weeks 25.2 Gy flank XRT; 10.8-Gy boost for lymph node involvement or gross disease
III AV × 4 weeks Low risk AV × 27 weeks None
Intermediate risk Postoperative tumor volume > 500 mL* AV × 27 weeks v AVD × 27 weeks 14.4 Gy flank XRT; 10.8-Gy boost for lymph node involvement or gross disease
High risk DCBE × 34 weeks 25.2 Gy flank XRT; 10.8-Gy boost for lymph node involvement or gross disease
IV AVD × 6 weeks Low risk Lung nodule CR AVD × 27 weeks Flank XRT for local stage III
No lung CR DCBE × 34 weeks 15 Gy lung; flank XRT for local stage III
Intermediate risk Lung nodule CR AVD × 27 weeks Flank XRT for local stage III
No lung CR DCBE × 34 weeks 15 Gy lung; flank XRT for local stage III
High risk§ Lung nodule CR DCBE × 34 weeks Flank XRT for local stage II/III
No lung CR DCBE × 34 weeks 15 Gy lung; flank XRT for local stage II/III

Abbreviations: AV, dactinomycin/vincristine; AVD, dactinomycin/vincristine/doxorubicin (cumulative doxorubicin dose, 250 mg/m2 for stage I to III; 300 mg/m2 for stage IV); CR, complete response; DCBE, doxorubicin/cyclophosphamide/carboplatin/etoposide (cumulative doxorubicin dose, 300 mg/m2 for stage IV); SIOP, International Society of Pediatric Oncology; XRT, radiation therapy.

*

In Germany, tumor volume > 500 mL after preoperative chemotherapy without stromal or epithelial predominance was assigned to high-risk histology.

CR attained by chemotherapy or metastasectomy.

Metastatic sites other than lung were also irradiated; XRT dose varied according to metastatic site.

§

Metastatic site irradiation regardless of response was mandated for anaplastic histology primary tumors and not if the abdominal tumor was blastemal type, unless anaplastic features were also found.