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Clinical grouping of rhabdomyosarcoma by the intergroup rhabdomyosarcoma study group (IRSG)

Clinical grouping of rhabdomyosarcoma by the intergroup rhabdomyosarcoma study group (IRSG)
Clinical group Extent of disease/surgical result
I A Localized tumor, confined to site of origin, completely resected
B Localized tumor, infiltrating beyond site of origin, completely resected
II A Localized tumor, gross total resection, but with microscopic residual disease
B Locally extensive tumor (spread to regional lymph nodes), completely resected
C Locally extensive tumor (spread to regional lymph nodes), gross total resection, but microscopic residual disease
III A Localized or locally extensive tumor, gross residual disease after biopsy only
B Localized or locally extensive tumor, gross residual disease after major resection (≥50 percent debulking)
IV* Any size primary tumor, with or without regional lymph node involvement, with distant metastases, irrespective of surgical approach to primary tumor
* Although current Children's Oncology Group (COG) trials include all patients with metastatic disease in the high-risk category, selected patients with favorable site, histology/molecular features (embryonal or alveolar, and FOXO1 fusion-negative), and age (under age 10) with limited metastases may have better outcomes with VAC therapy (vincristine, actinomycin D, and cyclophosphamide) or intensified treatments such as those in the completed ARST0431 protocol.
Modified from Crist W, Gehan EA, Ragab AH, et al. The Third Intergroup Rhabdomyosarcoma Study. J Clin Oncol 1995; 13:610; and Crist W, Garnsey L, Beltangady M, et al. Prognosis in children with rhabdomyosarcoma: A report of the intergroup rhabdomyosarcoma studies I and II. Intergroup Rhabdomyosarcoma Committee. J Clin Oncol 1990; 8:443.
Graphic 59875 Version 5.0

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