Current management of pediatric soft tissue sarcomas

Table 1 Intergroup rhabdomyosarcoma study pretreatment staging and clinical grouping classification

Stage	Sites	T1	Size2	N3	M4
1	Orbit, head and neck (excluding parameningeal), genitourinary tract (non-bladder, non-prostate), biliary tract	T1 or T2	a or b	N0 or N1 or Nx	M0
2	Bladder.Prostate, extremity, parameningeal, others (trunk, retroperitoneum, etc.)	T1 or T2	a	N0 or Nx	M0
3	Bladder.Prostate, extremity, parameningeal, others (trunk, retroperitoneum, etc.)	T1 or T2	a	N1	M0
			b	N0 or N1 or Nx	M0
4	Any sites	T1 or T2	a or b	N0 or N1	M1
Clinical group	Description
I	Localized disease, completely resected
II	Grossly resected tumor with evidence of regional spread
	IIA: Grossly resected tumor with microscopic residual disease
	IIB: Involved regional nodes completely resected with no microscopic residual disease
	IIC: Involved regional nodes grossly resected with evidence of microscopic residual disease
III	Incomplete resection with gross residual disease after biopsy or after gross or major resection of the primary tumor
IV	Distant metastatic disease present at diagnosis

¹T: Tumor; T1: Confined to the anatomic origin; T2: Extension and/or fixation to surrounding tissue;

²Size, a: ≤ 5 cm; b: > 5 cm;

³N: Regional nodes; N0: Regional nodes not clinically involved; Nx: Clinical status of regional nodes unknown; N1: Regional nodes clinically involved;

⁴M: Metastasis; M0: No distant metastasis; M1: Distant metastasis present (includes positive cytology in pleural, peritoneal or cerebrospinal fluid).