Clinicopathologic variables associated with a good histological response to
primary chemotherapy in Ewing's sarcoma are identified. The histological r
esponse to preoperative chemotherapy in 243 cases of Ewing's sarcoma treate
d with neoadjuvant chemotherapy was analyzed in relation to different clini
copathological features (sex and age of the patients. tumor size, serum lac
tate dehydrogenase (LDH) levels, tumor site) and to the type and schedule o
f anticancer drugs delivered preoperatively according to three consecutive
chemotherapy regimens. A higher rate of good responses was achieved with th
e use of ifosfamide and dactinomycin in addition to a conventional three-dr
ug VAC regimen, suggesting that these drugs should be included from the beg
inning in neoadjuvant regimens for the treatment of Ewing's sarcoma. The an
alysis of event-free survival in 158 patients with a 4-year minimum follow-
up confirmed that histological response to preoperative chemotherapy is a r
eliable predictor of outcome in Ewing's sarcoma.