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一名42岁女性的前上纵隔副神经节瘤:诊断与治疗挑战——病例报告

Anterosuperior mediastinal paraganglioma in a 42-Year-old woman: a diagnostic and therapeutic challenge-a case report.

作者信息

Wang Zheng, Zong Wenkang, Liang Shuo, Zhou Fang, Lu Xike, Sun Daqiang

机构信息

Department of Thoracic Surgery, Tianjin Chest Hospital Affiliated to Tianjin University, Tianjin, China.

Department of Pathology, TTianjin Chest Hospital Affiliated to Tianjin University, Tianjin, China.

出版信息

J Cardiothorac Surg. 2025 Apr 7;20(1):182. doi: 10.1186/s13019-024-03283-9.

Abstract

BACKGROUND

Paragangliomas are rare neuroendocrine tumors predominantly located within the adrenal gland. Extra-adrenal paragangliomas, particularly those in the anterosuperior mediastinum, are exceedingly rare and pose significant diagnostic and therapeutic challenges due to their complex anatomical location.

CASE DESCRIPTION

A 42-year-old woman was found to have an anterosuperior mediastinal mass during a routine health screening. Enhanced chest computed tomography (CT) revealed an ovoid, low-density mass intricately associated with major vascular structures including the superior vena cava, brachiocephalic trunk, left common carotid artery, aortic arch, right anonymous vein, and right subclavian artery. Despite significant intraoperative blood loss of 2000 ml, the mass was successfully excised with meticulous surgical technique and effective hemostasis. Histopathological examination showed a classic Zellballen pattern with chief cells and sustentacular cells embedded in a vascular-rich stroma. Immunohistochemistry confirmed the tumor's chromaffin nature, with chief cells testing positive for CD56, Synaptophysin, and Chromogranin A, and sustentacular cells positive for S100 protein, consistent with a diagnosis of paraganglioma. The patient's postoperative recovery was uneventful, and she was discharged one week after surgery.

CONCLUSIONS

This case highlights the essential role of comprehensive preoperative imaging and the necessity for interdisciplinary surgical expertise in managing complex mediastinal paragangliomas. Advanced surgical techniques and careful intraoperative management are paramount to achieving successful outcomes. Appropriate imaging modalities and auxiliary laboratory tests are crucial for early detection of recurrences in these rare tumors.

摘要

背景

副神经节瘤是一种罕见的神经内分泌肿瘤,主要位于肾上腺。肾上腺外副神经节瘤,尤其是位于前上纵隔的副神经节瘤,极为罕见,因其解剖位置复杂,在诊断和治疗上具有重大挑战。

病例描述

一名42岁女性在常规健康筛查中发现前上纵隔有一肿块。胸部增强计算机断层扫描(CT)显示一个椭圆形低密度肿块,与包括上腔静脉、头臂干、左颈总动脉、主动脉弓、右无名静脉和右锁骨下动脉在内的主要血管结构紧密相连。尽管术中失血达2000毫升,但通过精细的手术技术和有效的止血措施,肿块被成功切除。组织病理学检查显示典型的Zellballen模式,主细胞和支持细胞嵌入富含血管的基质中。免疫组织化学证实了肿瘤的嗜铬性质,主细胞对CD56、突触素和嗜铬粒蛋白A检测呈阳性,支持细胞对S100蛋白呈阳性,符合副神经节瘤的诊断。患者术后恢复顺利,术后一周出院。

结论

本病例突出了术前全面影像学检查的重要作用以及跨学科手术专业知识在处理复杂纵隔副神经节瘤中的必要性。先进的手术技术和术中仔细管理对于取得成功结果至关重要。适当的影像学检查方法和辅助实验室检查对于早期发现这些罕见肿瘤的复发至关重要。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/85ce/11974066/af4a94e3d4f2/13019_2024_3283_Fig1_HTML.jpg

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