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一名青少年中伪装成腹腔内脓肿的腹膜后尤因肉瘤:一项罕见的诊断挑战。

Retroperitoneal Ewing Sarcoma Masquerading as an Intraabdominal Abscess in a Teenager: A Rare Diagnostic Challenge.

作者信息

Hahn Daniel, Velasquez Lauren, Muller Nickolas, Lawless Margaret

机构信息

Internal Medicine, Touro College of Osteopathic Medicine, New York, USA.

Pathology, St. Mary's General Hospital, Passaic, USA.

出版信息

Cureus. 2025 Mar 19;17(3):e80853. doi: 10.7759/cureus.80853. eCollection 2025 Mar.

Abstract

Retroperitoneal Ewing sarcoma is a rare and aggressive extraskeletal variant of Ewing sarcoma, often presenting with nonspecific symptoms that can delay diagnosis. We discuss the case of a 19-year-old male who presented to the emergency room with progressive left abdominal and flank pain. Imaging revealed a retroperitoneal, necrotic mass and biopsy with immunohistochemistry confirmed retroperitoneal Ewing sarcoma with NKX2.2 and CD99 positivity. Fluorescence in situ hybridization (FISH) analysis showed that 98% of cells had an EWSR1 gene rearrangement. For treatment, the patient underwent a CT-guided biopsy and chemotherapy was initiated.  The sometimes-insidious progression of retroperitoneal Ewing sarcoma, coupled with the retroperitoneal space's ability to accommodate tumors without significant symptoms, often leads to late-stage diagnosis and poor prognosis. This report emphasizes the importance of early recognition and highlights the need for further research into optimal diagnostic protocols and treatment strategies.

摘要

腹膜后尤因肉瘤是尤因肉瘤一种罕见且侵袭性的骨外变体,常表现为非特异性症状,可导致诊断延迟。我们讨论了一名19岁男性的病例,该患者因进行性左腹部和侧腹痛就诊于急诊室。影像学检查发现腹膜后有一个坏死肿块,活检及免疫组化证实为腹膜后尤因肉瘤,NKX2.2和CD99呈阳性。荧光原位杂交(FISH)分析显示98%的细胞存在EWSR1基因重排。治疗方面,患者接受了CT引导下活检并开始化疗。腹膜后尤因肉瘤有时进展隐匿,加上腹膜后间隙能够容纳肿瘤而无明显症状,常导致晚期诊断和预后不良。本报告强调了早期识别的重要性,并突出了对最佳诊断方案和治疗策略进行进一步研究的必要性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b236/12007975/53fa283ed5a3/cureus-0017-00000080853-i01.jpg

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