Baggenstos Martin, Chew Emily, Butman John A, Oldfield Edward H, Lonser Russell R
Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, Maryland 20892-1414, USA.
J Neurosurg. 2008 Aug;109(2):313-7. doi: 10.3171/JNS/2008/109/8/0313.
Hemangioblastomas are frequently associated with peritumoral edema caused by extravasation of plasma ultrafiltrate through permeable neoplastic vessels. The authors report the clinical and imaging findings in a 62-year-old man with von Hippel-Lindau disease who presented with rapid (within 24 hours) loss of color vision and nearcomplete loss of left eye vision (acuity too poor to test). Serial MR imaging demonstrated a stable vascular tumor in the medioinferior aspect of the left optic nerve, associated with progressive edema extending from the nerve through to the bilateral optic radiations. Complete resection of the lesion was performed via an extended transsphenoidal approach, and histological examination confirmed the lesion was a hemangioblastoma. Postoperatively, the patient recovered color vision and had improvement in visual acuity (20/320). Serial imaging in this unique case captured the progressive extravasation of peritumoral edema that tracked and defined the parallel white matter tracts of first- and second-order neurons of the optic system, causing vision loss. Tumor resection led to resolution of the edema and improvement in visual function.
血管母细胞瘤常伴有瘤周水肿，这是由于血浆超滤液通过渗透性肿瘤血管外渗所致。作者报告了一名62岁患有冯·希佩尔-林道病的男性患者的临床和影像学表现，该患者出现快速（24小时内）色觉丧失和左眼视力几乎完全丧失（视力太差无法检测）。系列磁共振成像显示左侧视神经中下方面有一个稳定的血管性肿瘤，伴有从神经延伸至双侧视辐射的进行性水肿。通过扩大经蝶窦入路对病变进行了完整切除，组织学检查证实病变为血管母细胞瘤。术后，患者恢复了色觉，视力也有所改善（20/320）。该独特病例的系列成像捕捉到了瘤周水肿的进行性外渗，其追踪并界定了视系统一级和二级神经元的平行白质束，导致视力丧失。肿瘤切除导致水肿消退和视觉功能改善。
