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腹胀、免疫阳性及侵袭性:一例罕见的肾上腺尤因肉瘤

Abdominal distention, immunopositivity, and aggressive nature: A rare Ewing's sarcoma of the adrenal gland.

作者信息

Ali Anam, Shahbaz Usman, Nadeem Arsalan, Umar Sumayya, Ahmed Abdullah

机构信息

Department of Medicine, Jinnah Hospital, Allama Shabbir Ahmad Usmani Road, Lahore, Punjab, Pakistan.

Department of Medicine, Allama Iqbal Medical College, Allama Shabbir Ahmad Usmani Road, Lahore, Punjab, Pakistan.

出版信息

Radiol Case Rep. 2024 Feb 27;19(5):1935-1939. doi: 10.1016/j.radcr.2024.02.024. eCollection 2024 May.

Abstract

This case report describes a 32-year-old Pakistani male patient with an Ewing sarcoma (ES) of the adrenal gland. Presenting complaints were abdominal distention, pain, low-grade fever, and weight loss. Initial studies, including imaging and tumor markers, ruled out any other possible origins of the mass. A percutaneous biopsy verified the tumor's neuroendocrine origin. Extensive involvement of nearby anatomical structures was discovered through exploratory laparotomy, rendering total resection difficult. Based on the presence of malignant, round, blue cells that were positive for specific immunostaining markers, the histopathology report supported the diagnosis of an ES with a staging of T3N0M0. Chemotherapy, in accordance with the VAC-IE protocol, was administered after debulking surgery. Subsequent imaging and close monitoring revealed no metastatic or residual tumors. Adrenal ES is an uncommon, aggressive tumor that mandates prompt diagnosis and management. This case report highlights the value of early detection and multimodal therapy in enhancing patient outcomes for this rare malignancy.

摘要

本病例报告描述了一名32岁的巴基斯坦男性患者,患有肾上腺尤因肉瘤(ES)。主要症状为腹胀、疼痛、低热和体重减轻。包括影像学和肿瘤标志物在内的初步检查排除了肿块的任何其他可能来源。经皮活检证实肿瘤起源于神经内分泌。通过剖腹探查发现附近解剖结构广泛受累,难以进行根治性切除。根据特定免疫染色标志物呈阳性的恶性圆形蓝色细胞的存在,组织病理学报告支持ES的诊断,分期为T3N0M0。减瘤手术后,按照VAC-IE方案进行化疗。随后的影像学检查和密切监测显示没有转移瘤或残留肿瘤。肾上腺ES是一种罕见的侵袭性肿瘤,需要及时诊断和治疗。本病例报告强调了早期发现和多模式治疗对于改善这种罕见恶性肿瘤患者预后的价值。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/dec5/10915781/390a6ec607fe/gr1.jpg

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