冯·希佩尔-林道病中的脊髓软脑膜血管母细胞瘤病：磁共振成像及病理表现
Spinal leptomeningeal hemangioblastomatosis in von Hippel-Lindau disease: magnetic resonance and pathological findings.
作者信息
Bakshi R, Mechtler L L, Patel M J, Lindsay B D, Messinger S, Gibbons K J
机构信息
Dent Neurologic Institute, Millard Fillmore Health System, Department of Neurology, University of Buffalo, State University of New York Medicine and Biomedical Sciences, USA.
出版信息
J Neuroimaging. 1997 Oct;7(4):242-4. doi: 10.1111/jon199774242.
A 55-year-old man with von Hippel-Lindau disease presented with quadriparesis. Multiple enhancing cervical and thoracic spinal masses were seen on magnetic resonance imaging (MRI). A rim of diffuse, nodular enhancement linking all of the discrete masses was apparent on the surface of the cervical and thoracic regions of the cord. Surgical exploration revealed multiple extramedullary-intradural and intramedullary masses, extending to and infiltrating the cord; the leptomeninges contained numerous small tumor seeds at several levels. The excised spinal masses were diagnosed as capillary hemangioblastomas, which infiltrated the pia mater. Diffuse, intense, spinal leptomeningeal enhancement on MRI associated with multiple hemangioblastomas has not been previously reported and may be referred to as spinal "leptomeningeal hemangioblastomatosis."
一名患有冯希佩尔-林道病的55岁男性出现四肢瘫痪。磁共振成像（MRI）显示颈椎和胸椎有多个强化肿块。在脊髓颈段和胸段表面可见一圈弥漫性、结节状强化，连接所有离散肿块。手术探查发现多个髓外硬膜内和髓内肿块，延伸至并浸润脊髓；软脑膜在多个层面含有大量小肿瘤结节。切除的脊髓肿块被诊断为毛细血管型血管母细胞瘤，浸润软脑膜。MRI上与多个血管母细胞瘤相关的弥漫性、强烈的脊髓软脑膜强化此前未见报道，可能被称为脊髓“软脑膜血管母细胞瘤病”。
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