Background: Desmoplastic small round-cell tumor (DSCRT) in adults is an extremely rare (age-adjusted incidence 0.3 per million) and aggressive sarcoma with limited data for optimal management. Patients and Methods: Retrospective analysis of patients with DSCRT diagnosis (2010DSCRT diagnosis ( -2020 was performed following Institutional Review Board approval. The follow-up period was from pathological diagnosis to the last patient contact. Endpoints were type of response and duration of response. Results: In the current analysis, first-line treatment in all cases was vincristine, anthracycline, and cyclophosphamide alternating with ifosfamide and etoposide (VAC-IE) with 100% response for a mean duration of 9.8 (range=5-12) months. Patients received 1-4 subsequent lines of therapy. All patients received temozolomide with irinotecan (50% partial response, duration 8-9 months). Two patients that underwent consolidative cytoreductive surgery with hyperthermic intraperitoneal chemotherapy had a longer survival (30.6 vs. 11.2 months). Patients suffered 100% mortality with a median survival was 17.8 (range=11.2-30.6) months. Conclusion: While aggressive multimodality treatment is always warranted for DSCRT, the options are limited by the multicentric presentation, short-lived initial response and lack of established subsequent therapy portending a poor prognosis. Consolidative cytoreductive surgery following first-line therapy may improve survival.Desmoplastic small round-cell tumor (DSCRT) in adults is a rare sarcoma predominantly affecting young adults, with an 3859 This article is freely accessible online.

e16214 Background: Pancreatic ductal adenocarcinoma (PDAC) cancer portends a poor prognosis with a high case-fatality rate. Venous thromboembolism (VTE) is a common complication in PDAC, due expression of tissue factor on neoplastic cells. Per most recent guidelines, anticoagulation for primary prophylaxis (PPx) of VTE is to be considered based on the Khorana score. The purpose of this study is to identify patients with PDAC and compare the outcomes of those receiving anticoagulation for primary prophylaxis versus those who did not. Methods: We performed a retrospective review of all patients diagnosed with PDAC from 2017-2019 at Allegheny General Hospital. Data analysis was completed using IBM SPSS v23. Summary statistics were presented using percentages for categorical variables and medians with interquartile ranges for continuous variables. Univariable and multivariable logistic regression models were used to study the predictors of developing VTE, expressed as odds ratio (OR). Means and percentages were compared using t-test and Chi-square test, respectively. Results: Out of 171 patients, 121 received treatment at our institution and were included in the analysis. Median age was 69 years, 54 (45%) were male. The majority were white (88%). Only 92 patients had complete data regarding VTE PPx and events. 26 patients (28.2%) developed at least one VTE event. Out of 92 patients, 12 (13%) were on one form of VTE PPx and 1 had a contraindication to VTE PPx. One patient without VTE PPx died secondary to VTE. Of the patients on VTE PPx, only 3 (30%) experienced bleeding events. Seven patients were on VTE PPx prior to diagnosis of malignancy. 58 patients (63%) had a Khorana score of 2; 21 (23%) patients had a Khorana score of 3; 9 (10%) patients had a Khorana score of 4; and 4 (4%) patients had a Khorana score of 5. On univariable analysis, St. IV disease (OR 3.34, CI.22-8.40, P < 0.01), planned surgery (OR 0.26, CI 0.12-0.55, p < 0.01) and unresectable disease (OR 3.19, 95% CI 1.17-8.70, p 0.02) were predictors of developing VTE. On multivariate analysis, total pancreatectomy had a predictive association in the development of VTE (OR 5.01, 95% CI 1.65-23.60, p < 0.01). There was no difference in average time to progression (months) between patients who had VTE vs those who did not (12.5 vs 11.4, p 0.60). Conclusions: Our analysis indicate that we may be underutilizing Khorana score to prescribe VTE PPx in patients with PDAC. Additionally, patients undergoing total pancreatectomy demonstrated predictive association with the development of VTE. With these findings, we believe education and increased awareness of the importance of the Khorana score may decrease the morbidity and mortality associated with VTE in patients with PDAC. The use of electronic medical records to prompt physicians to calculate the Khorana score will also likely be a helpful tool.