Analysis of clinical features and prognostic factors of desmoplastic small round cell tumor
10.3969/j.issn.1674-8115.2018.03.008
- Author:
Rong-Xuan ZHU
1
Author Information
1. Department of Oncology, Shanghai General Hospital, Shanghai Jiao Tong University School of Medicine
- Publication Type:Journal Article
- Keywords:
Chemotherapy;
Desmoplastic small round cell tumor (DSRCT);
Prognosis factor;
Surgery
- From:
Journal of Shanghai Jiaotong University(Medical Science)
2018;38(3):281-287
- CountryChina
- Language:Chinese
-
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.
