Gani Faiz, Goel Utkarsh, Canner Joseph K, Meyer Christian F, Johnston Fabian M
Department of Surgery, Johns Hopkins University School of Medicine, Baltimore, Maryland.
Department of Surgery, Johns Hopkins Surgery Center for Outcomes Research, Johns Hopkins University School of Medicine, Baltimore, Maryland.
J Surg Oncol. 2019 Jun;119(7):880-886. doi: 10.1002/jso.25426. Epub 2019 Mar 6.
Because of the rarity of desmoplastic small round cell tumors (DSRCT), there is a lack of data describing patterns of care and survival for these patients. Using a national tumor registry, the current study sought to describe patterns of care and clinical outcomes for patients with DSCRT.
Data from the National Cancer Database were used to identify 491 patients aged 18 years or older diagnosed with DSRCT between 2004 and 2014. Multivariable Cox proportional hazards regression analysis was used to identify factors associated with overall survival (OS).
Among all patients, 41.2% (n = 200), underwent surgical resection of their primary tumor, chemotherapy was administered to 86.5% (n = 415) of patients, while radiation therapy was administered to 13.0% (n = 63) of patients. Over the study, 69.7% of patients died with a median OS of 25.9 months (interquartile range [IQR]: 22.7-27.5); 1-, 3-, and 5-year OS were 78.6%, 32.3%, and 18.4%, respectively. On multivariable analysis, stage IV disease (Hazard Ratio [HR] = 2.12, 95% CI: 1.41-3.18), receipt of surgery (HR = 0.68, 95% CI: 0.50-0.91), chemotherapy (HR = 0.52, 95% CI: 0.35-0.78), or radiation therapy (HR = 0.55, 95% CI: 0.33-0.92) were independently associated with OS.
Although receipt of multimodality treatment may lead to improved survival, further research and clinical trials are required to establish best practices for the care of DSRCT.
由于促结缔组织增生性小圆细胞肿瘤（DSRCT）较为罕见，缺乏关于这些患者的治疗模式和生存情况的数据。本研究利用国家肿瘤登记处的数据，试图描述DSCRT患者的治疗模式和临床结局。
使用国家癌症数据库的数据，确定了2004年至2014年间491例年龄在18岁及以上、诊断为DSRCT的患者。采用多变量Cox比例风险回归分析来确定与总生存期（OS）相关的因素。
在所有患者中，41.2%（n = 200）接受了原发肿瘤的手术切除，86.5%（n = 415）的患者接受了化疗，而13.0%（n = 63）的患者接受了放疗。在研究期间，69.7%的患者死亡，中位总生存期为25.9个月（四分位间距[IQR]：22.7 - 27.5）；1年、3年和5年总生存率分别为78.6%、32.3%和18.4%。多变量分析显示，IV期疾病（风险比[HR] = 2.12，95%置信区间：1.41 - 3.18）、接受手术（HR = 0.68，95%置信区间：0.50 - 0.91）、化疗（HR = 0.52，95%置信区间：0.35 - 0.78）或放疗（HR = 0.55，95%置信区间：0.33 - 0.92）与总生存期独立相关。
尽管接受多模式治疗可能会提高生存率，但仍需要进一步的研究和临床试验来确立DSRCT治疗的最佳实践。
