Abstract
Children with rhabdomyosarcoma who have localized gross residual disease after surgery (Group III disease) are at high risk for disease recurrence and death. In the first national study (IRS-I, 1972-78), 261 Gp III pts were treated with VACĀ± adriamycin for 2 yrs plus postoperative radiation. At 3 yrs, the relapse-free survival (RFS) rate was 60% and the overall survival rate was 57%. In IRS-II (1978-84), 370 pts were treated with intensive repetitive pulse VACĀ± adriamycin for 2 yrs and postoperative radiation. Pts. with cranial parameningeal (PM) tumors at risk of CNS extension received, in addition, cranial radiation plus intrathecal methotrexate, hydrocortisone and cytosine arabinoside. The 3-yr RFS and overall survival rates of 72% and 70% on IRS-II were superior (P < .002) to IRS-I results. The major reason for improved outcome was the superior results in PM pts who accounted for 37% of Gp III pts in both studies. The 3-yr RFS and overall survival rates for PM patients in IRS-II vs.-I were: 72% vs. 58% (p=.04); 69% vs. 52% (p=.002). Improved outcome for all other pts was less significant (IRS-II vs.-I: RFS-72% vs. 60%; overall survival - 68% vs. 58%; p=.08 for both). We conclude that CNS prophylaxis improves survival in pts. with PM tumors and that intensive chemotherapy for 2 yrs improves the outcome for all Gp III pts.
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Maurer, H., Raney, R., Ragab, A. et al. 924 IMPROVED SURVIVAL OF CHILDREN WITH GROUP III RHABDOMYOSARCOMA (NON-SPECIAL PELVIC SITES). Pediatr Res 19, 264 (1985). https://doi.org/10.1203/00006450-198504000-00954
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DOI: https://doi.org/10.1203/00006450-198504000-00954