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伴有双侧嗜铬细胞瘤和肝囊肿的小脑脊髓血管母细胞瘤:一种罕见病例。

Cerebellospinal hemangioblastoma with bilateral pheochromocytoma and hepatic cyst: A rare entity.

作者信息

Bhuyan Mrinal, Dutta Deep, Baishya Basanta Kumar, Hussain Zakir

机构信息

Department of Neurosurgery, Gauhati Medical College, Guwahati, Assam, India.

出版信息

Asian J Neurosurg. 2016 Jul-Sep;11(3):311. doi: 10.4103/1793-5482.179644.

Abstract

A 45-year-old gentleman presented with headache, dizziness, and unsteadiness of gait. On imaging, he was found to have a cerebellar and a spinal intramedullary hemangioblastoma. To rule out Von Hippel-Lindau (VHL) disease, we did screening of the whole abdomen with ultrasonography followed by contrast-enhanced computed tomography of the abdomen, and surprisingly, the patient was having bilateral adrenal pheochromocytoma and a hepatic cyst. A diagnosis of VHL disease was made on this basis. The patient was operated and he made a very good recovery. We are reporting this interesting case because of its typical findings and its rarity. Disease management and review of literature were also discussed at the end.

摘要

一位45岁男性因头痛、头晕及步态不稳前来就诊。影像学检查发现他患有小脑及脊髓髓内血管母细胞瘤。为排除冯·希佩尔-林道(VHL)病,我们先对全腹部进行了超声筛查,随后进行了腹部增强计算机断层扫描,令人惊讶的是,该患者患有双侧肾上腺嗜铬细胞瘤及一个肝囊肿。基于此做出了VHL病的诊断。患者接受了手术,恢复情况良好。我们报告这一有趣病例是因其典型表现及罕见性。最后还讨论了疾病管理及文献回顾。

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