Presumptive Extra-Skeletal Ewing Sarcoma of the Jejunum with Disseminated Metastases
After 18 months of ripretinib therapy, a color ultrasound scan at another hospital in April 2023 revealed an irregular, isoechoic/hypoechoic mass (60 mm × 33 mm × 20 mm in size) with clear borders in the subcutaneous muscle layer of the left posterior thoracic wall.
A 63‑year‑old man presented with a large jejunal mass and extensive intra‑abdominal metastases. Core biopsy revealed a small round cell tumour positive for CD99 and nuclear NKX2.2, supporting a presumptive diagnosis of extraskeletal Ewing sarcoma. Surgical resection was not feasible; palliative chemotherapy was recommended but never initiated. This case highlights diagnostic challenges of jejunal Ewing sarcoma and the potential for cancer‑associated thromboembolic events.
This rare presentation underscores the difficulty of diagnosing extraskeletal Ewing sarcoma in adults with gastrointestinal symptoms, the importance of a comprehensive immunohistochemical and molecular work‑up, and the risk of malignant hypercoagulability leading to ischemic stroke.
Evidence level: Klinikai vizsgálat. Formális klinikai vizsgálati eredmény.
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