Xing Pu-yuan, Shi Yuan-kai, Feng Feng-yi, Qin Yan, Liu Peng
Department of Medical Oncology, Cancer Institute & Hospital, Chinese Academy of Medical Sciences, Peking Union Medical College, Beijing, China.
Zhonghua Zhong Liu Za Zhi. 2010 Feb;32(2):139-42.
To investigate the clinical characteristics and treatment of desmoplastic small round cell tumor.
Five patients with DSRCT were diagnosed and treated in our Hospital from January 1999 to May 2009. Forty-eight cases with complete clinical data were collected and reviewed from 23 published reports. Therefore totally 53 patients with DSRCT were analysed. The survival rate was calculated by Kaplan-Meier method and compared by log-rank test.
The median age of all cases was 23 (1.5 - 66) years old at the time of diagnosis. 75.5% of patients were male. The most common presenting complaint was intra-abdominal mass or pain (77.4%). In 46 patients (86.8%), the primary tumor was located in the abdomen or pelvis. Fifteen (28.3%) had positive lymph nodes or distant parenchymal metastases. The median follow-up was 1.8 years (range, 0.1 - 10.0 years). The overall 1-, 3- and 5-year survivals were 45.8%, 20.8% and 5.7%, respectively. Forty-seven patients underwent surgery. Complete tumor resection was significantly correlated with long survival. The 1- and 3-year survival rates were 70.5% and 53.7% in patients treated with complete tumor resection compared to 37.2% and 4.8% in the incomplete tumor resection cohort (P = 0.0020). Thirty-four patients received chemotherapy and the 1- and 3-year survival rates were 60.1% and 35.2%, respectively, however, only 29.7% and 12.7% in patients without chemotherapy (P = 0.0396). Twelve patients had radiotherapy and the 1- and 3-year survival rates were 75.0% and 38.9%, respectively, compared with 36.9% and 14.8% in those without radiotherapy (P = 0.0314).
Complete tumor resection results in improved survival in patients with DSRCT. Chemotherapy and radiotherapy correlate with improved patient outcome. Multimodal therapy may improve the survival in patients with DSRCT.
探讨促结缔组织增生性小圆细胞肿瘤的临床特征及治疗方法。
1999年1月至2009年5月，我院诊治了5例促结缔组织增生性小圆细胞肿瘤患者。从23篇已发表的报告中收集并回顾了48例具有完整临床资料的病例。因此，共分析了53例促结缔组织增生性小圆细胞肿瘤患者。采用Kaplan-Meier法计算生存率，并通过对数秩检验进行比较。
所有病例诊断时的中位年龄为23岁（1.5 - 66岁）。75.5%的患者为男性。最常见的主诉是腹部肿块或疼痛（77.4%）。46例患者（86.8%）的原发性肿瘤位于腹部或骨盆。15例（28.3%）有阳性淋巴结或远处实质转移。中位随访时间为1.8年（范围0.1 - 10.0年）。总体1年、3年和5年生存率分别为45.8%、20.8%和5.7%。47例患者接受了手术。肿瘤完全切除与长期生存显著相关。肿瘤完全切除患者的1年和3年生存率分别为70.5%和53.7%，而肿瘤切除不完全组分别为37.2%和4.8%（P = 0.0020）。34例患者接受了化疗，1年和3年生存率分别为60.1%和35.2%，然而，未接受化疗的患者分别为29.7%和12.7%（P = 0.0396）。12例患者接受了放疗，1年和3年生存率分别为75.0%和38.9%，而未接受放疗的患者分别为36.9%和14.8%（P = 0.0314）。
肿瘤完全切除可提高促结缔组织增生性小圆细胞肿瘤患者的生存率。化疗和放疗与改善患者预后相关。多模式治疗可能提高促结缔组织增生性小圆细胞肿瘤患者的生存率。
