Spinal Hemangioblastomas in Von Hippel-Lindau Disease Mimicking a Spinal Dural Arteriovenous Fistula

Abstract

Introduction Spinal hemangioblastomas (HBs) are rare tumors that are closely associated with a diagnosis of Von Hippel-Lindau disease (VHL). A tissue diagnosis is needed to make the diagnosis of HB because many other entities can present similarly, which may require different management strategies. We present the first case in the literature of multiple intramedullary spinal HBs and VHL that mimicked a spinal dural arteriovenous fistula (dAVF) on radiological imaging. Material and Method Case illustration and literature review. Results A 60-year-old man was referred to our clinic for evaluation of progressive radiculomyelopathy and evidence of a T10 dAVF on MRI and MRA. MRI demonstrated perimedullar hypervascularity dorsal to the spinal cord from T10 to L1, and associated T2 signal change within the spinal cord. Prominent radicular arteries were identified at the exiting right T10 nerve root sleeve which was suspicious for the fistulous point. The patient underwent a T10-T12 laminoplasty for intradural exploration and possible ligation of the fistula; however, intraoperative findings included multiple small intra- and extramedullary vascular nodules along the spinal cord. Six lesions were biopsied, revealing a diagnosis of HB and VHL. Indocyanine green angiography (ICG) was used to rule out a concomitant dAVF. He is being managed expectantly and undergoing routine screening for VHL. He was discharged on postoperative day 3, and he reported symptom improvement at 2-week follow-up. Conclusion The present case is the first reported in the literature to describe multiple spinal HBs, in the setting of VHL, that appeared to be dAVF preoperatively. The patient's late clinical presentation, the greater degree of associated hypervascularity, and atypical vascular morphology were unique features, which highlight the diversity of this disease. Disclosure of Interest None declared References Wanebo JE, Lonser RR, Glenn GM, Oldfield EH. 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