Presumptive Ewing Sarcoma of the Jejunum Presenting with Metastatic Disease: A Case Report and Focused Narrative Review

Objective: To report a rare case of presumptive Ewing sarcoma family tumor of the jejunum with disseminated metastases and cancer-associated stroke in an older adult, and to review the diagnostic challenges in differentiating jejunal EES from other gastrointestinal small round cell tumors. Material and Methods: Clinical-imaging-pathological, and laboratory records from a 63-year-old man with presumptive jejunal Ewing sarcoma family tumor, disseminated intra‑abdominal metastases, and cancer‑associated ischemic stroke compiled into a detailed case report. A targeted literature search of PubMed and Google Scholar using combinations of "jejunal Ewing sarcoma", "small bowel extraskeletal Ewing sarcoma", and "gastrointestinal Ewing sarcoma", and reference lists of key articles were manually searched to capture additional reports. Results: Presenting with subacute abdominal pain, distension, weight loss, low-grade fever, and exertional dyspnea. CT showed a large circumferential jejunal mass with extensive hepatic, peritoneal, and pelvic metastases. Core biopsy was consistent with a malignant small round cell tumor positive for CD99 and nuclear NKX2.2, supporting a presumptive diagnosis of Ewing sarcoma family tumor, but extended immunohistochemistry and EWSR1 FISH/RT‑PCR were not available. He also developed an acute right fronto-parieto-occipital infarct with isolated left wrist drop and profound hypercoagulability with cancer-associated stroke considered the most likely etiology. Palliative chemotherapy was recommended, but no treatment was ultimately administered because he left against medical advice, and long-term follow-up was unavailable. Conclusion: This case report and focused narrative review illustrate a rare presentation of presumptive jejunal Ewing sarcoma family tumor with disseminated metastases and associated stroke in an older adult, emphasize diagnostic pitfalls versus GNET, clear cell sarcoma, GIST, lymphoma, and highlight central but incomplete role of CD99/NKX2.2 without molecular confirmation in resource‑limited settings.