Rhabdomyosarcoma prognostic stratification

Prognosis (EFS)	Stage	Clinical group	Site	Size	Age	FOXO1 fusion status*	Mets	Nodes
Excellent (>85%) Low risk subset A	1	I	Favorable	a or b	<21	Negative	M0	N0
	1	II	Favorable	a or b	<21	Negative	M0	N0
	1	III	Orbit only	a or b	<21	Negative	M0	N0
	2	I	Unfavorable	a	<21	Negative	M0	N0 or NX
	1	II	Favorable	a or b	<21	Negative	M0	N1
Very good (70 to 85%) Low risk subset B	1	III	Orbit only	a or b	<21	Negative	M0	N1
	1	III	Favorable, excluding orbit	a or b	<21	Negative	M0	N0 or N1 or NX
	2	II	Unfavorable	a	<21	Negative	M0	N0 or NX
	3	I or II	Unfavorable	a	<21	Negative	M0	N1
	3	I or II	Unfavorable	b	<21	Negative	M0	N0 or N1 or NX
Good (50 to 70%) Intermediate risk	2	III	Unfavorable	a	<21	Negative	M0	N0 or NX
	3	III	Unfavorable	a	<21	Negative	M0	N1
	3	III	Unfavorable	b	<21	Negative	M0	N0 or N1 or NX
	1, 2, 3	I, II, III	Favorable or unfavorable	a or b	<21	Positive	M0	N0 or N1 or NX
	4	IV	Favorable or unfavorable	a or b	<10	Negative	M1	N0 or N1 or NX
Poor (<30%) High risk	4	IV	Favorable or unfavorable	a or b	≥10	Negative	M1	N0 or N1 or NX
Poor (<30%) High risk	4	IV	Favorable or unfavorable	a or b	<21	Positive	M1	N0 or N1 or NX

The risk group descriptions in this table are based upon the results of historically completed trials using the EFS estimates of the individual patient groups. Current Children's Oncology Group (COG) protocols can deviate from theses definitions for protocol purposes (refer to text).

EFS: event-free survival; favorable site: orbit/eyelid, head and neck (excluding parameningeal), genitourinary (not bladder or prostate), and biliary tract; unfavorable site: bladder, prostate, extremity, parameningeal, trunk, retroperitoneal, pelvis, other; a: tumor size 5 cm in diameter; b: tumor size >5 cm in diameter; N0: regional nodes clinically not involved; N2: regional nodes clinically involved; NX: node status unknown; M0: no distant metastases; M1: distant metastases present.
* For the management of tumors with alveolar histology and negative for FOXO1 fusion, refer to UpToDate content on treatment of rhabdomyosarcoma.

Adapted with permission from: Raney RB, Anderson JR, Barr FG, et al. Rhabdomyosarcoma and undifferentiated sarcoma in the first two decades of life: A selective review of Intergroup Rhabdomyosarcoma Study Group Experience and rationale for Intergroup Rhabdomyosarcoma Study V. J Pediatr Hematol Oncol 2001; 23:215. Copyright © 2001 Lippincott Williams & Wilkins.

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Rhabdomyosarcoma prognostic stratification