Clinical characteristics and outcomes of pediatric patients with desmoplastic small round cell tumor
Clinical characteristics and outcomes of pediatric patients with desmoplastic small round cell tumor
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DOI：
10.4081/rt.2016.6145
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发表时间：
2016-01-01
期刊：
影响因子：
0.9
通讯作者：
DuBois, Steven G.
中科院分区：
文献类型：
--
作者：
Bent, Melissa A.;Padilla, Benjamin E.;DuBois, Steven G.
结缔组织增生小圆细胞瘤（DSRCT）是一种罕见的恶性肿瘤，通常影响儿童和年轻成人患者。关于儿童DSRCT的临床特征数据有限。我们选择的患者年龄为0-21岁。1991-2011年流行病学和最终结果项目。我们使用Kaplan-Meier方法估计总生存率，并使用log rank检验比较结果。95例儿童患者的中位年龄为15.3岁（范围：0-21岁）。大多数肿瘤起源于腹部和骨盆（84.4%），大多数患者有远处转移（72.6%）。少数患者接受放射治疗（34%）。5年总生存率较差（18.1%；95%可信区间为10.1-27.9%）。放射治疗与更高的生存率相关。儿童DSRCT患者有显著的疾病负担。儿童的预后很差，尽管选择接受放射治疗的患者似乎改善了生存率。
Desmoplastic small round cell tumor (DSRCT) is a rare malignancy that typically affects pediatric and young adult patients. There are limited data on the clinical features of pediatric DSRCT. We selected patients aged 0-21 years reported to the Surveillance. Epidemiology and End Results Program from 1991-2011. We estimated overall survival using Kaplan-Meier approaches and compared outcomes using the log rank test. The median age of the 95 pediatric patients was 15.3 years (range: 0-21). The majority of tumors originated in the abdomen and pelvis (84.4%) and the majority of patients had distant metastasis (72.6%). A minority of patients received radiation (34%). Overall survival at 5 years was poor (18.1%; 95% confidence interval 10.1-27.9%). Radiation therapy was associated with superior survival. Pediatric patients with DSRCT have significant disease burden. Outcomes for children are poor, though patients selected for radiation appear to have improved survival.