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).

Table 2 Risk group stratification for rhabdomyosarcoma according to the International Rhabdomyosarcoma Study

Risk group	Histology	Pretreatment stage	Clinical group
Low (subset 1)	Embryonal	1	I, II
		1	III (orbit)
		2	I, II
Low (subset 2)		1	III (non-orbit)
		3	I, II
Intermediate	Embryonal	2, 3	III
	Alveolar	1, 2, 3	I, II, III
High	Any	4	IV

Table 3 Risk stratification in nonrhabdomyosarcoma soft tissue sarcoma and treatment proposal according to the Children's Oncology Group (NCT00346164)

Risk group	Factors				Proposed treatment
	Grade	Size	Stage	Initial resectability
Low	Low	Any	Nonmetastatic	Gross resection	Observation
	High	< 5 cm	Nonmetastatic	Without microscopic margins	Observation
	High	< 5 cm	Nonmetastatic	With microscopic margin	Adjuvant radiation therapy
Intermediate	High	> 5 cm	Nonmetastatic	Gross resection	Adjuvant chemotherapy and radiation therapy
	High	> 5 cm	Nonmetastatic	Unresected	Neoadjuvant chemoradiotherapy, surgery, adjuvant chemotherapy with or without radiation therapy
High	Low	Any	Metastatic	Gross resection	Observation
	High	Any	Metastatic	Gross resection	Adjuvant chemotherapy and radiation therapy
	High	Any	Metastatic	Unresected	Neoadjuvant chemoradiotherapy, surgery, adjuvant chemotherapy with or without radiation therapy