Childhood Rhabdomyosarcoma Treatment (PDQ®): Treatment - Health Professional Information [NCI] - Stage Information
Table 3. Soft Tissue Sarcoma Committee of the Children's Oncology Group: Surgical-pathologic Group System
|I||Approximately 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.|
|II||Approximately 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.|
|III||Approximately 48%||Localized tumor, incompletely removed with gross, residual disease after: (a) biopsy only,or(b) gross major resection of the primary tumor (>50%).|
|IV||Approximately 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.
Table 4. Soft Tissue Sarcoma Committee of the Children's Oncology Group: Rhabdomyosarcoma Risk Group Classification
|Low risk||Embryonal||1||I, II, III|
|Embryonal||2, 3||I, II|
|Intermediate risk||Embryonal||2, 3||III|
|Alveolar||1, 2, 3||I, II, III|
|High risk||Embryonal or Alveolar||4||IV|
Since 2006, patients with undifferentiated sarcomas are treated on the COG-STS protocol for nonrhabdomyosarcomatous soft tissue sarcoma. Refer to the PDQ summary on Childhood Soft Tissue Sarcoma for more information.
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