Jeong Hyehyun, Hong Yong Sang, Kim Young-Hoon, Kim Chan Wook, Song Si Yeol, Song Joon Seon, Cho Kyung-Ja, Kim Jeong Eun, Ahn Jin-Hee
Department of Oncology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Republic of Korea.
Division of Kidney and Pancreas Transplantation, Department of Surgery, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Republic of Korea.
Clin Med Insights Oncol. 2021 Feb 17;15:1179554920987107. doi: 10.1177/1179554920987107. eCollection 2021.
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.
We reviewed medical records of 14 patients with pathologically confirmed DSRCT at Asan Medical Center between 2004 and 2018.
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; = .024) and surgery with intended complete resection (43.5 vs 20.1 months; = .027).
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.
多模式治疗是促结缔组织增生性小圆细胞肿瘤（DSRCT）的标准治疗方法；然而，许多患者被诊断为无法手术的疾病，这使得化疗成为唯一的治疗选择。关于姑息化疗有效性的数据有限，尤其是在一线治疗后使用时。在此，我们评估了接受多线化疗的DSRCT患者的临床结局。
我们回顾了2004年至2018年间在峨山医学中心14例经病理证实为DSRCT患者的病历。
诊断时的中位年龄为25岁，男性占患者的92.9%。所有患者初诊时均为无法手术的疾病，并接受化疗作为初始治疗。4例患者（28.6%）接受了手术治疗，其中1例实现了完全切除。中位总生存期（OS）为23.9个月，1年、2年和3年生存率分别为92.9%、48.6%和19.5%。在接受一线（N = 14）、二线（N = 10）和三线（N = 8）化疗的患者中，中位疾病进展时间分别为9.9个月、3.5个月和2.5个月，疾病控制率分别为100%、88.9%和75.0%。单变量分析中与较长OS相关的因素是初诊时转移部位≤2个（27.0对14.7个月；P = 0.024）以及预期完全切除的手术（43.5对20.1个月；P = 0.027）。
尽管晚期DSRCT在一线治疗后最初可能对化疗有反应，但随着疾病进展，反应的持续时间会缩短。应做出以姑息治疗为重点的个体化治疗决策。
