Background: A multimodal approach is the standard treatment for desmoplastic small round cell tumor (DSRCT); however, many patients are diagnosed with inoperable disease, which leaves chemotherapy as the only treatment option. There are limited data on the effectiveness of palliative chemotherapy, especially when used after first-line treatment. Here, we evaluated the clinical outcomes of patients with DSRCT treated with multiple lines of chemotherapy. Methods: We reviewed medical records of 14 patients with pathologically confirmed DSRCT at Asan Medical Center between 2004 and 2018. Results: The median age at diagnosis was 25, with males comprising 92.9% of patients. All patients had inoperable disease at presentation and received chemotherapy as the initial treatment. Four patients (28.6%) were treated with surgery, and complete resection was achieved in 1 patient. Median overall survival (OS) was 23.9?months, and 1-, 2-, and 3-year survival rates were 92.9%, 48.6%, and 19.5%, respectively. In patients receiving first- (N = 14), second- (N = 10), and third-line (N = 8) chemotherapy, median time-to-progression was 9.9, 3.5, and 2.5?months, respectively, and the disease control rates were 100%, 88.9%, and 75.0%, respectively. Factors associated with longer OS in the univariable analysis were ?2 metastatic sites at presentation (27.0 vs 14.7?months; P = .024) and surgery with intended complete resection (43.5 vs 20.1?months; P = .027). Conclusions: Although advanced DSRCT may initially respond to chemotherapy after first-line treatment, the response becomes less durable as the disease progresses. Individualized treatment decisions focused on palliation should be made.
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机译:背景:多峰方法是脱塑料小圆形细胞肿瘤(DSRCT)的标准治疗;然而,许多患者被诊断出患有无法操作的疾病,这将化疗作为唯一的治疗方案。有限的数据数据有关姑息化疗的有效性,特别是在第一线治疗后使用时。在这里,我们评估了用多种化疗治疗DSRCT患者的临床结果。方法:在2004年至2018年间,我们审查了14例病理证实DSRCT的病症14例病理学记录。结果:诊断的中位年龄为25岁,患者患有92.9%的患者。所有患者在介绍和接受化疗作为初始治疗的情况下均可予以操作。用手术治疗四名患者(28.6%),1例患者达到完全切除。中位数总生存(OS)为23.9?月,1 - ,2-和3年生存率分别为92.9%,48.6%和19.5%。在接受第一 - (n = 14)的患者中,第二 - (n = 10)和第三线(n = 8)化疗,分别为9.9,3.5和2.5?数月,以及疾病控制率分别为100%,88.9%和75.0%。与不可变化分析中的更长OS相关的因素是介绍的2个转移性位点(27.0 vs 14.7?月份; p = .024)和预期切除的手术(43.5 Vs 20.1?月份; p = .027)。结论:虽然先进的DSRCT在一线治疗后最初对化疗进行响应,但随着疾病的进展,响应变得更耐用。应制定专注于痛苦的个性化治疗决策。
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