Spinal Hemangioblastomas in Von Hippel-Lindau Disease Mimicking a Spinal Dural Arteriovenous Fistula
Authors: Kalyvas J. , Change S. 
Published: May 2014

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. The natural history of hemangioblastomas of the central nervous system in patients with von Hippel-Lindau disease. J Neurosurg 2003;98(1):82–94 Maher ER, Yates JR, Harries R, et al. Clinical features and natural history of von Hippel-Lindau disease. Q J Med 1990;77(283):1151–1163 Murota T, Symon L. Surgical management of hemangioblastoma of the spinal cord: a report of 18 cases. Neurosurgery 1989;25(5):699–707, discussion 708 Neumann HP. Basic criteria for clinical diagnosis and genetic counselling in von Hippel-Lindau syndrome. Vasa 1987;16(3):220–226 Yasargil MG, Antic J, Laciga R, de Preux J, Fideler RW, Boone SC. The microsurgical removal of intramedullary spinal hemangioblastomas. Report of twelve cases and a review of the literature. Surg Neurol 1976; (3):141–148 Ammerman JM, Lonser RR, Dambrosia J, Butman JA, Oldfield EH. Long-term natural history of hemangioblastomas in patients with von Hippel-Lindau disease: implications for treatment. J Neurosurg 2006;105(2):248–255 Killory BD, Nakaji P, Maughan PH, Wait SD, Spetzler RF. Evaluation of angiographically occult spinal dural arteriovenous fistulae with surgical microscope-integrated intraoperative near-infrared indocyanine green angiography: report of 3 cases. Neurosurgery 2011;68(3):781-787, discussion 787 Wong GK, Zhu XL, Ng HK, et al. Hemangioblastoma of filum terminale associated with arteriovenous shunting. Surg Neurol 2007;68(2):211–214, discussion 214–215 Lonser RR, Weil RJ, Wanebo JE, DeVroom HL, Oldfield EH. Surgical management of spinal cord hemangioblastomas in patients with von Hippel-Lindau disease. J Neurosurg 2003;98(1):106–116 Tamura Y, Hirota Y, Miyata S, Yamada Y, Tucker A, Kuroiwa T. The use of intraoperative near-infrared indocyanine green videoangiography in the microscopic resection of hemangioblastomas. Acta Neurochir (Wien) 2012;154(8):1407–1412, discussion 1412 Cramer F, Kimsey MW. The cerebellar hemangioblastomas; review of fifty-three cases with special reference to cerebellar cysts and the association of polycythemia. AMA Arch Neurol Psychiatry 1952;67(2):237–252 Silver ML, Hennigar G. Cerebellar hemangioma (hemangioblastoma); a clinicopathological review of 40 cases. J Neurosurg 1952;9(5):484–494 Asthagiri AR, Mehta GU, Zach L, et al. Prospective evaluation of radiosurgery for hemangioblastomas in von Hippel-Lindau disease. Neuro-oncol 2010;12(1):80–86 Mehta GU, Asthagiri AR, Bakhtian KD, Auh S, Oldfield EH, Lonser RR. Functional outcome after resection of spinal cord hemangioblastomas associated with von Hippel-Lindau disease. J Neurosurg Spine 2010;12(3):233–242 Koch C. Spinal dural arteriovenous fistula. Curr Opin Neurol 2006;19(1):69–75 Jellema K, Canta LR, Tijssen CC, van Rooij WJ, Koudstaal PJ, van Gijn J. Spinal dural arteriovenous fistulas: clinical features in 80 patients. J Neurol Neurosurg Psychiatry 2003;74(10):1438–1440 Logue V. Angiomas of the spinal cord: review of the pathogenesis, clinical features, and results of surgery. J Neurol Neurosurg Psychiatry 1979;42(1):1–11 Steinmetz MP, Chow MM, Krishnaney AA, et al. Outcome after the treatment of spinal dural arteriovenous fistulae: a contemporary single-institution series and meta-analysis. Neurosurgery 2004;55(1):77–87, discussion 87–88 Conway JE, Chou D, Clatterbuck RE, Brem H, Long DM, Rigamonti D. Hemangioblastomas of the central nervous system in von Hippel-Lindau syndrome and sporadic disease. Neurosurgery 2001;48(1):55-62, discussion 62–63 Wang C, Zhang J, Liu A, Sun B. Surgical management of medullary hemangioblastoma. Report of 47 cases. Surg Neurol 2001;56(4):218-226, discussion 226–227 Zhou LF, Du G, Mao Y, Zhang R. Diagnosis and surgical treatment of brainstem hemangioblastomas. Surg Neurol 2005;63(4):307-315, discussion 315–316