Abdominopelvic Imaging: GastrointestinalMalignant Gastrointestinal Neuroectodermal Tumor: A New Kid on the Block?Morani, Ajaykumar C. MD∗; Ramani, Nisha S. MD†; Yedururi, Sireesha MD∗; Prasad, Srinivasa R. MD∗ Author Information From the ∗Department of Abdominal Radiology, The University of Texas MD Anderson Cancer Center †Department of Pathology, Michael E. DeBakey VA Medical Center, Houston, TX. Received for publication January 10, 2022; accepted April 21, 2022. Correspondence to: Ajaykumar C. Morani, MD, Department of Abdominal Radiology, The University of Texas MD Anderson Cancer Center, 1400 Pressler St, Unit 1473, Houston, TX 77030-4009 (e-mail: [email protected]). The authors declare no conflict of interest. Journal of Computer Assisted Tomography: 9/10 2022 - Volume 46 - Issue 5 - p 676-681 doi: 10.1097/RCT.0000000000001350 Buy Metrics Abstract Also referred to as “osteoclast-rich, clear cell sarcoma–like tumor of the gastrointestinal tract (CCSLGT),” malignant gastrointestinal neuroectodermal tumor is a newly described, rare, aggressive sarcoma that commonly arises in the small bowel, stomach, and colon. Histogenesis is likely from an autonomous nervous system–related primitive cell of neural crest origin. The hallmark genetic finding of EWS-CREB1 or EWS-ATF1 fusion transcripts clinches the diagnosis. Annular constrictive lesions tend to be smaller, show homogenous contrast enhancement on computed tomography, and may present with bowel obstruction. Larger, expansile masses tend to be exophytic and show heterogeneous contrast enhancement. Surgical resection is the mainstay of treatment. Frequent recurrences, metastases, and death from disease in 75% of patients portend a poor prognosis. Targeted chemotherapy based on specific tumor pathways is being developed. Copyright © 2022 Wolters Kluwer Health, Inc. All rights reserved.