Time to Diagnosis of Ewing Tumors in Children and Adolescents Is Not Associated With Metastasis or Survival: A Prospective Multicenter Study of 436 Patients
Menée en France à partir de données portant sur 436 jeunes patients atteints d'un sarcome d'Ewing traité entre 1988 et 2000 (âge inférieur à 21 ans), cette étude prospective multicentrique met en évidence l'absence d'association entre le délai pour diagnostiquer la maladie et la survenue de métastases ou la survie à long terme
Purpose : The time to diagnosis (TtD) of Ewing tumors is one of the longest among pediatric tumors. Its precise consequences, however, have not been studied well. We analyzed the distribution of TtD for Ewing tumors in children and adolescents and its association with clinical features, tumor characteristics, surgical outcome, and long-term survival.
Patients and Methods : We analyzed prospectively collected data from two multicenter clinical trials of patients younger than 21 years old who had Ewing bone tumors treated in France between 1988 and 2000. Clinical and tumoral features, TtD, and outcome associations were studied by univariable and multivariable analyses.
Results : The median TtD for the 436 patients was 70 days (interquartile range, 27 to 146 days), with no significant decrease during the study period (P > .2). The factors associated with long TtD were older age and some tumor sites (pelvis, extremities of limbs). Increased tumor volume and decreased histologic response to chemotherapy were associated with long TtD on univariable analysis (P <.05) but not after adjustment. Presence of a nerve or spinal-cord compression at diagnosis, presence or site of metastasis, surgical treatment, mutilating surgery, complete resection, or survival were not associated with TtD.
Conclusion : TtD of Ewing tumors was long, especially for adolescents and for certain tumor sites, and did not improve over time. But TtD was not associated with metastasis, surgical outcome, or survival. These findings could be used to comfort parents at diagnosis and in expert testimony produced for malpractice claims.
Journal of Clinical Oncology , résumé, 2014