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Childhood Rhabdomyosarcoma Treatment (PDQ®): Treatment - Health Professional Information [NCI] - Stage Information

Table 2. Soft Tissue Sarcoma Committee of the Children's Oncology Group: Pretreatment Staging System

StageSites of Primary TumorT StageTumor SizeRegional Lymph NodesDistant Metastasis
N0 = absence of nodal spread; N1 = presence of regional nodal spread beyond the primary site; X = unknown N status; M0 = absence of metastatic spread; M1 = presence of metastatic spread beyond the primary site and regional lymph nodes; T1 = tumor confined to anatomic site of origin (noninvasive); T2a = tumor extension and/or fixation to surrounding tissue (invasive), tumor ≤5 cm in maximum diameter; T2b = tumor extension and/or fixation to surrounding tissue (invasive), tumor >5 cm in maximum diameter.
1Favorable sitesT1 or T2Any sizeN0 or N1 or NXM0
2Unfavorable sitesT1 or T2a, ≤ 5 cmN0 or NXM0
3Unfavorable sitesT1 or T2a, ≤ 5 cmN1M0
b, > 5 cmN0 or N1 or NX
4Any siteT1 or T2Any sizeN0 or N1 or NXM1

The IRS-I, IRS-II, and IRS-III studies prescribed treatment plans based on the Surgical-pathologic Group system. In this system, Groups are defined by the extent of disease and by the completeness or extent of initial surgical resection after pathologic review of the tumor specimen(s). The definitions for these Groups are shown in Table 3 below.[7,8]

Table 3. Soft Tissue Sarcoma Committee of the Children's Oncology Group: Surgical-pathologic Group System

GroupIncidenceDefinition
IApproximately 13%Localized tumor, completely removed with microscopically clear margins and no regional lymph node involvement. Lymph node biopsy or sampling is encouraged if lymph nodes are clinically or radiographically suspicious.
IIApproximately 20%Localized tumor, completely removed with: (a) microscopic disease at the margin, (b) regional disease with involved, grossly removed regional lymph nodes without microresidual disease,or(c) regional disease with involved nodes, grossly removed but with microscopic residual and/or histologic involvement of the most distal node from the primary tumor.
IIIApproximately 48%Localized tumor, incompletely removed with gross, residual disease after: (a) biopsy only,or(b) gross major resection of the primary tumor (>50%).
IVApproximately 18%Distant metastases are present at diagnosis. This category includes: (a) radiographically identified evidence of tumor spread,and(b) positive tumor cells in cerebral spinal fluid, pleural, or peritoneal fluids, or implants in these regions.

After patients are categorized by Stage and Surgical-pathologic Group, a Risk Group is assigned. This takes into account Stage, Group, and histology. Patients are classified for protocol purposes as having a low risk, intermediate risk, or high risk of disease recurrence.[9,10] Treatment assignment is based on Risk Group, as shown in Table 4. To be designated as alveolar, the tumor must have greater than 50% alveolar elements; if the alveolar component is 50% or less, the tumor is considered embryonal.

1|2|3

WebMD Public Information from the National Cancer Institute

Last Updated: February 25, 2014
This information is not intended to replace the advice of a doctor. Healthwise disclaims any liability for the decisions you make based on this information.
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