Nonrhabdomyosarcoma in children: prognostic factors influencing survival

In approximately 90% of children with nonrhabdo soft tissue sarcoma (NRSTS) local control can be obtained by either surgery alone or with supplemental radiation. The disease-free survival is, however, only about 50%. To determine factors influencing overall survival, we undertook a retrospective review of the 154 children with NRSTS. We used the American Joint Committee on Cancer staging system (1988), using invasiveness, size, and a Pediatric Oncology Group grading system. Using this system we documented that 72 (46%) were noninvasive (T1) lesions. Of these 72 patients 50 (70%) were < or = 5 cm A, and only 9 (18%) had G3 lesions. Overall 7/72 (10%) have died. In contrast to the 82 patients with invasive (T2) lesions, 65 (79%) were > 5 cm (B), with approximately 80% G3. Of all T2 lesions, 56/82 were G3 (65%). Here, overall 58/82 (70%) have died, primarily because 49/58 had G3 lesions. Important prognostic factors include primarily G3 lesion 52/71 (73%) mortality, as compared to 13/83 (15%), G1-2 dying. Children with invasive lesions tended to have larger tumors, 50/65, most of which were higher grades. We feel that patients with histologic grade G3 and T2 lesions should be enrolled in effective chemotherapy protocols.