Abeer Qasim, , Haozhe Sun, MD, Anil Dev, MD BronxCare Health System, Bronx, NY
Introduction: Ewing sarcoma (ES) is a rare and highly aggressive tumor composed of small, round cells, showing varying neuroectodermal differentiation and marked by the presence of EWSR1-ETS fusion proteins.The Ewing's sarcoma family of tumors (EFTs) includes Ewing's sarcoma, extra-skeletal Ewing's sarcoma, Askin's tumor, and peripheral primitive neuroectodermal tumor, all sharing similar histological, immunohistochemical, and chromosomal translocation characteristics.Metastasis in ES typically occurs in the chest wall, head and neck, paravertebral region, and retroperitoneum. However, rare cases have been reported in abdominal soft tissues. This case study discusses a 56-year-old man diagnosed with ES with metastasis to the stomach, an exceptionally uncommon site for ES metastasis.
Case Description/Methods: A 56-year-old man with advanced HIV/AIDS and Ewing sarcoma presented with left flank pain. Examination revealed costovertebral angle tenderness, a CD4 count of 21 cells/µL, and a viral load of 407,000 copies/mL. A CT scan showed left-sided pyelonephritis, pulmonary nodules, enlarged mediastinal lymph nodes, lytic bone lesions, and thickening of the gastric wall. An upper endoscopy revealed esophageal candidiasis and a gastric tumor, which biopsy confirmed as malignant, suggesting metastatic Ewing sarcoma. During his hospital stay, the patient developed a spontaneous left pneumothorax and, despite treatment, his condition worsened, leading to his death on the 22nd day of hospitalization.
Discussion:
Ewing Sarcoma (ES) is a rare, aggressive bone tumor common in individuals aged 10-19 but also observed in those under 10 and over 30. It is associated with familial cancer syndromes like Li-Fraumeni Syndrome and TP53 mutations. Immunodeficiency, such as from advanced HIV/AIDS, increases the risk of ES, as seen in a 56-year-old patient with a CD4 count of 21 who had extensive metastases. Diagnosis involves pathology and immunohistochemistry, revealing small, round blue cells and positive staining for vimentin or CD99. Treatment typically includes multimodal chemotherapy, surgery, radiation, and emerging therapies like stem cell transplantation and targeted treatments. Despite treatment advances, metastatic ES remains challenging to cure, necessitating ongoing research for better therapies.
Figure: endoscopy revealing gastric tumor was discovered in the cardia.
Disclosures:
Abeer Qasim indicated no relevant financial relationships.
Haozhe Sun indicated no relevant financial relationships.
Anil Dev indicated no relevant financial relationships.
Abeer Qasim, , Haozhe Sun, MD, Anil Dev, MD. P1629 - Stomach-Bound: Ewing Sarcoma's Unexpected Metastasis, ACG 2024 Annual Scientific Meeting Abstracts. Philadelphia, PA: American College of Gastroenterology.
