Desmoplastic Small Round Cell Tumors of the Gastrointestinal Tract

Simple SummaryDesmoplastic small round cell tumors (DSRCTs) are rare, aggressive malignant neoplasms that typically affect the abdominal region. The challenge in diagnosing those tumors is rendered to their histopathologic resemblance to other small round cell tumors, such as Ewing sarcoma and rhabdomyosarcoma. Our review aims to provide a better understanding of the key histomorphologic and genetic features that make DSRCTs unique, including specific gene fusions. We highlight the importance of ancillary tests, including immunohistochemical staining and molecular profiling, in making an accurate diagnosis. By improving the way these tumors are identified and treated, we hope this review will help guide both pathologists and clinicians and improve outcomes for patients with DSRCTs.