Journal of Clinical Oncology, Vol 16, 3736-3743, Copyright © 1998 by American Society of Clinical Oncology
Ewing's family of tumors in adults: multivariate analysis of survival and long-term results of multimodality therapy in 182 patients
K Fizazi, N Dohollou, JY Blay, S Guerin, A Le Cesne, F Andre, P Pouillart, T Tursz and BB Nguyen
Department of Medical Oncology, Institut Gustave-Roussy, Villejuif, France. fizazi@igr.fr
PURPOSE: To assess the outcome and the prognosis of adults with a neoplasm
related to the Ewing's sarcoma family of tumors. PATIENTS AND METHODS: The
outcomes of 182 consecutive patients older than 15 years with Ewing's
sarcoma or related neoplasms managed from 1982 to 1992 were reviewed,
without any selection according to primary tumor site or disease extension.
RESULTS: Of 182 patients, 53 had evidence of metastases at presentation
(29%). Tumor size was greater than 10 cm in 70 patients (41%). With a
median follow-up duration of 66 months, the 5- year overall survival (OS)
rate was 41%. In patients with localized disease, 5-year OS rate was 54%
and 5-year progression-free survival (PFS) rate, 43%. Late relapses after 5
years accounted for 9% of relapses. Metastasis at presentation (P =
.00001), pelvic primary lesion (P = .0025), and tumor size greater than 10
cm (P = .004) were independent prognostic factors for survival. Five-year
OS was 67% in patients with nonpelvic tumors < or = 10 cm, 52% in those
with pelvic tumors less than 10 cm or extrapelvic tumors > or = 10 cm,
16% in those with pelvic tumors greater than 10 cm, and 9% in those with
metastasis (P = .00001). CONCLUSION: Based on our experience and a review
of the literature, we concluded that the natural history and the prognosis
of the Ewing's family of tumors in adults are not different from that found
in children. A greater tumor bulk in adults may explain the less favorable
prognosis previously reported by others. Outcome could be adequately
monitored by a simple prognostic index.
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