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Association of Time to Diagnosis of ESFT with Tumor Origin and Location, Metastasis, and Survival: A Single Institutional Study of 374 Cases.

Journal of clinical oncology(2014)

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摘要
e21502 Background: Ewing’s sarcoma family of tumors (ESFT) is a rare aggressive bone tumor of childhood with poor outcome. In developing countries most of ESFT patients have delayed presentation. We studied determinants of duration between first symptoms and diagnosis (time to diagnosis: TtD) of ESFT and its impact on disease extension at diagnosis and outcome. Methods: This is a single institutional study of ESFT patients treated between June 2003 and November 2011. Relations between TtD and baseline demographics and treatment were evaluated. Further, the median time duration was used for categorization to evaluate its impact on survival. Results: The median TtD of the 374 cases was 5.28 months (interquartile range 3.4 months - 8.47 months). The factors associated with a longer TtD were older age (mean 7.8 months for children >15 years vs 6 for those less than 15 years, p=0.009), tumor localization (7.8 months for axial primary vs 6.2 months for appendicular location, p=0.003) and tumor origin (mean 7.1 months for skeletal primary vs 5.4 months for soft tissue origin, p=0.02), but not with tumor volume. We did not find any significant association between TtD and year of diagnosis, sex, tumor diameter, systemic symptom, presence of local pain or swelling, presence or type of metastasis, therapy-related toxicity, amenability for surgical resection or survival [5-years overall survival and EFS of 45.6±4.9% and 31.2±4.1% for patients with TtD shorter than the median (5 months) vs 33.1±5.1% (p=0.37) and 20.7±3.6% (p=0.32), respectively]. Conclusions: TtD of ESFT were unusually long in our cohort. They were associated with older age group, tumor origin and tumor location pain but not with metastasis or survival. These findings should be used to inform care givers at diagnosis and educate primary care physicians to decrease the TtD.
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