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A case of submandibular desmoplastic small round cell tumor: Diagnostic and management approaches to an atypical presentation of a rare tumor

To the editor: Desmoplastic small round cell tumor (DSRCT) is a rare malignant neoplasm first described in 1989 by Gerald and Rosai characterized by a translocation of t(11;22)(p13;q12) leading to a EWS-WT1 fusion protein.1 Typically affecting pediatric and young adult patients, the overwhelming majority of cases originate in the abdomen and pelvis with coincidentdistantmetastases.2 Outcomeshave traditionally been dismal in this population, with five-year overall survival rates on the order of only 18% to 25% even with aggressive multimodal treatment regimens.3,4 To someextent, this is drivenbydifficulty achievingmicroscopically negative local control due to the frequently widespread