›› 2018, Vol. 38 ›› Issue (3): 281-.doi: 10.3969/j.issn.1674-8115.2018.03.008

• Original article (Clinical research) • Previous Articles     Next Articles

Analysis of clinical features and prognostic factors of desmoplastic small round cell tumor

ZHU Rong-xuan1 , WANG Jian2 , ZHU Li-fei1 , WANG Hong-xia1   

  1. 1. Department of Oncology, Shanghai General Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai 201600, China; 2. Department of Pathology, Fudan University Shanghai Cancer Center, Shanghai 200032, China
  • Online:2018-03-28 Published:2018-05-03
  • Supported by:
    National Natural Science Foundation of China, 81772802, 81628011; Key Project of Science and Technology Committee of Shanghai Municipality, 17JC1404400; Natural Science Foundation of Shanghai, 16ZR1427400

Abstract: Objective · To analyze the clinical features and prognostic factors of desmoplastic small round cell tumor (DSRCT). Methods · Clinical datum of 140 patients with DSRCT published from Nov. 2003 to Jul. 2012 were collected and studied retrospectively by searching Medline and Embase databases. The observation indicators were progression-free survival (PFS) or overall survival (OS). Survival rates were calculated using the Kaplan-Meier method and compared between groups using a log-rank test. Multivariate analysis was performed using the Cox model to determine the prognostic factors. Results · Patient median age was (23.2±12.7) years (range 4-74 years, the ratio of male and female was 3.12:1). Frequent symptoms were abdominal pain (35.7%) and evidence of a palpable mass (20.0%). 106 cases tumors were in the abdominal or pelvic cavity, the remaining were extra-abdominal tumors. The frequency of patients receiving conventional chemotherapy, cytoreductive surgery, neoadjuvant chemotherapy, adjuvant chemotherapy or first-line chemotherapy was 76.4%, 52.1%, 17.1%, 47.9% and 38.6%, respectively. Some patients received adjuvant radiotherapy (17.1%), hyperthermic intraperitoneal chemotherapy (4.1%) and bone marrow transplantation (7.3%). By univariate analysis, male gender, absence of metastasis, effective cytoreductive surgery, chemotherapy and multimodal therapy were significant prognostic factors for prolonged OS (all P<0.05). Primary tumor site, extraabdominal tumors, absence of metastasis and effective cytoreductive surgery were associated with improved PFS (all P<0.05). Cox regression analysis showed effective cytoreductive surgery and chemotherapy were independent prognostic factors. Conclusion · Multimodal therapeutics that clear tumors by surgery, adjuvant therapy are favorable prognostic factors for improved survival level in DSRCT patients.

Key words: desmoplastic small round cell tumor (DSRCT), surgery, chemotherapy, prognosis factor