血管母细胞瘤显示的Von Hippel-Lindau综合征单侧嗜铬细胞瘤。

Q4 Medicine
Kathmandu University Medical Journal Pub Date : 2024-07-01
S Kc, H Poudyal, S Baral
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引用次数: 0

摘要

Von Hippel-Lindau (VHL)综合征的特点是肿瘤良恶性兼有,典型表现为小脑血管母细胞瘤、肾细胞癌(RCC)、嗜铬细胞瘤等其他可能相关的肿瘤包括胰腺囊肿;神经内分泌肿瘤;附睾囊肿和内淋巴囊肿瘤本病例报告详细介绍了一名29岁男性患者,他以头痛伴头晕和呕吐一个月的病史来到急诊科。头部计算机断层扫描(CT)和磁共振成像(MRI)提示为小脑血管母细胞瘤。进一步检查显示多发性肿瘤,包括单侧嗜铬细胞瘤和胰腺囊肿,这与von Hippel-Lindau (VHL)综合征的诊断一致。值得注意的是,患者的儿茶酚胺和香草扁桃酸(VMA)水平在正常范围内。患者接受了小脑血管母细胞瘤的手术治疗,术后恢复顺利。这个病例值得注意,因为它的特征性发现和罕见的条件。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Unilateral Pheochromocytoma in Von Hippel-Lindau Syndrome Revealed by a Hemangioblastoma.

Von Hippel-Lindau (VHL) syndrome is characterized by the occurrence of both benign and malignant tumors, with typical manifestations such as cerebellar hemangioblastoma, Renal cell carcinoma (RCC) and pheochromocytomas.1 Additional tumors that may be associated include pancreatic cysts; neuroendocrine tumours; epididymal cysts and endolymphatic sac tumors.1 This case report details a 29-year-old male who arrived at the Emergency Department (ED) with a one-month history of headaches accompanied by dizziness and vomiting. A Computed Tomography (CT) scan of the head followed by Magnetic Resonance Imaging (MRI) suggested cerebellar hemangioblastoma. Further workup showed multiple tumors, including unilateral pheochromocytoma and pancreatic cysts, which aligned with a diagnosis of von Hippel-Lindau (VHL) syndrome. Notably, the patient's catecholamine and vanillylmandelic acid (VMA) levels were within normal limits. The patient underwent surgical intervention for the cerebellar hemangioblastoma, and the postoperative recovery was uneventful. This case is noteworthy due to its characteristic findings and the rarity of the condition.

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来源期刊
Kathmandu University Medical Journal
Kathmandu University Medical Journal Medicine-Medicine (all)
CiteScore
0.70
自引率
0.00%
发文量
51
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