Pediatric nonrhabdomyosarcomas of the extremities. Influence of size, invasiveness, and grade on outcome.

Surgery alone or supplemental radiation is effective in local control in a majority of patients with nonrhabdomyosarcoma of the soft tissue. To determine the factors influencing subsequent survival, a retrospective review of 64 children with nonrhabdomyosarcoma of the soft tissue of the extremities was performed. Using the American Joint Committee on Cancer staging system, 32 patients had noninvasive T1 lesions, and 31 of these patients survived disease free. Twenty-two patients had lesions that were smaller than 5 cm; 19 of these lesions were grade G1-2. All 22 patients survived. Ten patients had tumors that were 5 cm or larger, and all were G1-2; nine of these patients survived. Thirty-two patients had invasive (T2) lesions; seven of these lesions were smaller than 5 cm, but four were G3. Only one patient survived. Eighteen (72%) of 25 lesions that were 5 cm or larger were G3. Fifteen (83%) of these 18 patients underwent relapse, with 12 patients dying of the disease. Important prognostic factors included G3 lesions, with a 72% (18 of 25 patients) failure rate, and T2 lesions, which were generally larger than 5 cm, of higher grade, and underwent relapse more frequently than T1 lesions. These patients with histologic G3 and T2 lesions may benefit from chemotherapy.