Abstract

To determine whether low-dose irradiation (i.e., approximately 40 Gy at 1.5-1.8 Gy/fraction), which is associated with > or = 90% local control in children with initially resected rhabdomyosarcoma and microscopic residual [Intergroup Rhabdomyosarcoma (IRS) group II disease], achieves comparable results in children with locally advanced rhabdomyosarcoma (IRS group III) left with microscopic disease after induction chemotherapy with or without delayed surgery. Among 103 patients entered on five successive studies between 1968 and 1991, 24 had evidence of microscopic residual disease after initial surgical resection (IRS group II) and received low-dose irradiation. Initial chemotherapy was used in 79 with IRS group III disease. In 28 of these 79 group III patients, chemotherapy alone (n = 16) or in combination with delayed surgery (n = 12) reduced disease to microscopic levels prior to the start of radiotherapy based upon which they received low-dose irradiation. All have a minimum 2-year follow-up and median age of 4 years. Primary tumor sites among the 24 with group II disease included: orbit (5), parameningeal (2), nonparameningeal head and neck (3), genitourinary: nonbladder/prostate (5), extremity (4), and other (5). Irradiation dose ranged from 32-50 Gy, with a median and modal dose of 40 Gy. Primary tumor sites among the 28 with group III disease selectively treated with low-dose irradiation included: orbit (1), parameningeal (6), nonparameningeal head and neck (4), genitourinary: bladder/prostate (12) and nonbladder/prostate (1), extremity (1), and other (3). Irradiation dose ranged from 33-52 Gy, with a median and modal dose of 40 Gy. Local disease control has been maintained in 23 of 24 patients (96%) with group II disease. Local control occurred in eight of nine (89%) group II patients receiving < 40 Gy and in all 15 receiving > or = 40 Gy (p = 0.26). Twenty (83%) are alive and free of disease. Twenty-two of the 28 patients (79%) with group III disease treated with low-dose irradiation have maintained continuous local control of disease which was not statistically different from the group II patients (p = 0.08). Local control occurred in 7 of 11 (64%) group III patients receiving < 40 Gy vs. 15 of 17 (88%) receiving > or = 40 Gy (p < = 0.14). Nineteen (68%) are alive and free of disease. Survival in these group III patients is significantly worse than that of the group II patients, with 19 (68%) alive and free of disease (p = 0.04). Children with locally advanced rhabdomyosarcoma (IRS group III) who have only microscopic disease after induction chemotherapy with or without delayed surgery have a high likelihood of achieving local control with low-dose irradiation. For this group, data suggest treatment to a dose level of at least 40 Gy.

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