Introduction: Gastrointestinal neuroectodermal tumors (GNETs) are rare but distinctive sarcomas that often arise from the small intestines. Case Description: We report a 24-year-old Filipino male who initially presented with abdominal pain; imaging showed an enhancing circumferential mass involving the jejunum along with a peripherally enhancing focus in the liver and several enlarged superior mesenteric lymph nodes. Discussion: Histopathologic evaluation of the tumor revealed a monomorphic population of epithelioid cells with pale eosinophilic to clear cytoplasm and round nuclei with open chromatin and occasional conspicuous nucleoli. The tumor cells assume various architectural patterns with admixed osteoclast-like giant cells. Immunohistochemistry studies showed diffuse positivity with SOX10 and S100 and negativity for HMB45. Fluorescence in situ hybridization demonstrated EWSR1 gene rearrangement confirming the diagnosis of GNET. Despite their relatively bland morphology, these tumors demonstrate aggressive behavior. Conclusion In light of more recent reports of similar neoplasms occurring in extraenteric sites, heightened clinical suspicion and familiarity with this peculiar malignancy is recommended.
Jejunal Neoplasms ; Neuroectodermal Tumors ; Sarcoma