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小儿多发性嗜铬细胞瘤围手术期管理的综合病例报告及文献综述

Comprehensive case report and literature review on perioperative management of multiple pheochromocytoma in a pediatric patient.

作者信息

Yu Shenghua, Zou Tianxiao, Wei Sisi, Yu Yani, Ding Guili

机构信息

Department of Anesthesiology, Shanghai Children's Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai, China.

Department of SICU, Shanghai Children's Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai, China.

出版信息

Front Pediatr. 2025 Feb 11;13:1439186. doi: 10.3389/fped.2025.1439186. eCollection 2025.

Abstract

Pheochromocytomas (PCCs) are rare neuroendocrine tumors originating from the adrenal medulla, particularly uncommon in pediatric populations. This case report presents a 12-year-old boy with a three-year history of fatigue and a one-year history of blurred vision, who was admitted with severe hypertension (230/200 mmHg). Abdominal CT imaging revealed bilateral adrenal masses measuring 40 mm on the left and 12 mm on the right. The diagnosis of pheochromocytoma was confirmed by elevated blood catecholamine metabolites. During perioperative preparation, the patient experienced complications, including polyuria, thrombosis, and infection, necessitating an emergency resection of the right adrenal tumor. This intervention led to a successful recovery. Subsequent genetic testing identified a mutation in the VHL gene. After further medical management, the left adrenal tumor was also successfully removed. At one-year follow-up, the patient remained normotensive with no signs of recurrence. This case emphasizes the critical role of genetic testing and cutious perioperative management in the treatment of pediatric pheochromocytoma.

摘要

嗜铬细胞瘤(PCCs)是起源于肾上腺髓质的罕见神经内分泌肿瘤,在儿科人群中尤为罕见。本病例报告介绍了一名12岁男孩,有三年疲劳病史和一年视力模糊病史,因严重高血压(230/200 mmHg)入院。腹部CT成像显示双侧肾上腺肿块,左侧40 mm,右侧12 mm。血儿茶酚胺代谢产物升高证实了嗜铬细胞瘤的诊断。在围手术期准备过程中,患者出现了包括多尿、血栓形成和感染在内的并发症,因此需要紧急切除右侧肾上腺肿瘤。该干预措施使患者成功康复。随后的基因检测发现VHL基因存在突变。经过进一步的医学治疗,左侧肾上腺肿瘤也被成功切除。在一年的随访中,患者血压保持正常,无复发迹象。本病例强调了基因检测和谨慎的围手术期管理在小儿嗜铬细胞瘤治疗中的关键作用。

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