Desmoplastic small round cell tumor (DSRCT) is a rare and high malignant soft tissue tumor with very poor prognosis.It usually occurs in the abdominopelvic cavity of adolescents and young males.DSRCT is prone to occur distant metastasis,mainly in the liver and lung.The histopathological manifestation is featured with nests of small round blue cells separated by desmoplastic stroma.DSRCT can co-express epithelial,neural and mesenchymal markers.The molecular characteristic of DSRCT is the production of EWSWT1 fusion protein via the translocation of chromosome t (11;22) (p13;q12).Treatments of DSRCT include radical resection or cytoreductive surgery,high intensity systemic chemotherapy,local radiotherapy and hyperthermic intraperitoneal chemotherapy.

Objective:To investigate the effects of different treatment modes on the survival of patients with non-small cell lung cancer (NSCLC) brain metastases, and to evaluate the clinical values of diagnosis-specific graded prognostic assessment (DS-GPA) model and graded prognostic assessment model for lung cancer using molecular markers (Lung-molGPA).Methods:The clinical data of 195 NSCLC patients with brain metastases treated in the Cancer Hospital of Shantou University Medical College from January 2011 to December 2015 were retrospectively analyzed, including 112 patients without brain metastasis (metachronous brain metastases) at the first diagnosis, and 83 patients with brain metastases at the first diagnosis (simultaneous brain metastases). The treatment modalities of brain metastases included single local cranial radiation, chemotherapy, target therapy and combined cranial radiation with chemotherapy or target therapy, chemotherapy plus target therapy, et al. Kaplan-Meier method was used for survival analysis, Cox regression method was used for univariate and multivariate survival analyses, and DS-GPA and Lung-molGPA models were used for survival analysis.Results:The median time to brain metastases in all patients was 14.1 months (95% CI 12.2-16.0 months). The median progression-free survival (PFS BM) time of all patients was 4.3 months (95% CI 3.4-5.2 months), and the median overall survival (OS BM) time of brain metastases was 6.7 months (95% CI 4.6-8.8 months). There was no difference in PFS BM and OS BM between patients with synchronous and metachronous brain metastases ( P = 0.446, P = 0.080). Receiving anti-tumor therapy, especially combining targeted therapy could improve median OS BM. Low Karnofsky score ( RR = 1.698, 95% CI 1.238-2.329, P = 0.001) and bone metastasis ( RR = 1.505, 95%CI 1.089-2.081, P = 0.013) were independent risk factors for the OS BM of NSCLC patients with brain metastases, and chemotherapy ( RR = 0.460, 95% CI 0.289-0.731, P = 0.001) and brain radiotherapy ( RR = 0.541, 95% CI 0.391-0.749, P < 0.01) were independent protective factors for the OS BM of NSCLC patients with brain metastases. The OS BM difference between patients grouped by DS-GPA and Lung-molGPA models was statistically significant (median OS BM time of patients with DS-GPA model 0.0-1.0, 1.5-2.0 and 2.5-3.0 points were 4.2, 9.4 and 10.9 months, respectively, P = 0.015; median OS BM time of patients with Lung-molGPA model 0.0-1.0, 1.5-2.0 and 2.5-3.0 points were 4.1, 8.7 and 13.0 months, respectively, P < 0.01). Conclusions:The prognosis of NSCLC patients with brain metastases is poor, and anti-tumor therapy can prolong their survival. High Karnofsky score, without bone metastasis, receiving chemotherapy or brain radiotherapy are independent good prognostic factors for NSCLC patients with brain metastases. Both DS-GPA and Lung-molGPA models can predict the survival of NSCLC patients with brain metastases.