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. 2019 Aug 27;8(14):6437–6448. doi: 10.1002/cam4.2504

Table 1.

Risk group assignment by risk group definition

Risk group assignment Risk group stratification definition

D‐series (1997‐2004)

ARST‐series (2004‐2013)

Low

D9602 (NCT00002995): (ERMS only)

Subset A:

Fav site, any size, Stage 1, Group I and II, N0;

Fav site, any size, Stage 1, Group III, N0, (orbit only);

Unfav site, ≤ 5cm, Stage 2, Group I, N0, Nx;

Therapy: VA x 45 wk

Subset B:

Fav site, any size, Stage 1, Group II, N1;

Fav site, any size, Stage 1, Group III, N1 (orbit only);

Fav site (except orbit), any size, Stage 1, Group III, N0, N1;

Unfav site, ≤5 cm, Stage 2, Group II, N0, Nx;

Unfav site, ≤5 cm with N1 or >5 cm any size, Stage 3, Group I/II, N0, Nx, N1

Therapy similar to D9803

ARST0331 (NCT00075582): (ERMS only)

Subset 1:

Stage 1, Group I and II, N0;

Stage 1, Group III, N0, Nx, (orbit only);

Stage 2, Group I, N0, Nx, and Group II;

Therapy: VAc x 4, VA x4

Subset 2:

Stage 1, Group III, N0, Nx, (non‐orbital);

Stage 3, Group I/II

Therapy VAc x 4, VA x 12

Intermediate

D9803 (NCT00003958)

Stage 1‐3, Group I‐III, ARMS;

Stage 2/3, Group III, ERMS;

Stage 4, Group IV, ERMS, <10 years

Therapy: VAC vs VAC/VTC

ARST0531 (NCT00354835)

Stage 2/3, Group III, ERMS;

Stage 1‐3, Group I‐III, ARMS

Therapy: VAc vs VAc/VI

High

D9802 (NCT00003955)

Stage 4, Group IV, except ERMS <10 y

Therapy: I ± V; VAC

ARST0431 (NCT00354744)

Stage 4, Group IV

Therapy: VDc/IE/I/VAc

Abbreviations: ARMS, alveolar rhabdomyosarcoma; ERMS, embryonal rhabdomyosarcoma; Fav, favorable; I, irinotecan; N0, No regional nodal involvement; N1, regional nodal involvement; Nx, nodal involvement unknown; Unfav, unfavorable; V, vincristine; VA, vincristine/actinomycin; VAc, vincristine, actinomycin/cyclophosphamide (dose 1.2 gm/m2); VAC, vincristine, actinomycin/cyclophosphamide (dose 2.2 gm/m2); VDc, vincristine/doxorubicin/cyclophosphamide; IE, Ifosfamide/etoposide; VTC, vincristine, topotecan, cyclophosphamide.