Prognostic Role of Ultrasound Imaging in Desmoplastic Small Round Cell Tumor: A Retrospective Study and Literature Review

Abstract

\n Background: Desmoplastic small round cell tumor (DSRCT) mostly arose in the abdominopelvic cavity is an aggressive sarcoma with poor prognosis. The purpose of this study is to analyze ultrasonographic performance in prediction of prognosis of DSRCT.Method: Between March 1999 and October 2019, a total of 27 patients with pathologically confirmed DSRCT in our hospital were conducted. Clinical and ultrasonographic characterizations, including age, sex, symptoms, tumor size, number, location, shape, margin, echogenicity, homogeneity, vascularity, and metastases were recorded and analyzed with overall survival (OS) by using univariate analysis.Result: Ultrasonographic performance of DSRCT were irregular (74%), posterior echo enhancement (63%), calcification (51.9%), and liquefaction (55.6%) in tumors companied with ascites (63%), hydronephrosis (59%) and metastases (63%). The median OS was 22 months (range 7–36 months) and 5-year OS rate was 19.6%. Using univariate analysis, a higher hazard ratio (HR) of mortality was associated with liquefaction (HR: 3.09, 95% CI:1.23-7.76, p=0.016) and bone metastasis (HR: 2.95, 95%CI: 1.16-7.49, p=0.023). According to a risk staging system developed by selected predictors (low = neither liquefaction nor bone involvement; moderate = either liquefaction or bone involvement; high = both liquefaction and bone involvement), 40%, 10%, and 0 of 3-year survival were found in the low, moderate, and high risk patients, respectively.Conclusion: Ultrasound imaging is effective to evaluate prognosis of DSRCT. Findings of liquefaction and bone involvement in the ultrasonography may predict a poor prognosis of DSRCT patients.