Imaging in Spinal Hemangioblastoma 

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Hemangioblastomas are vascular tumors that can be found throughout the neuraxis, primarily in the cerebellum and spinal cord. Hemangioblastomas of the spinal cord account for approximately 3% of all intramedullary spinal tumors. ^{[1, 2]} These tumors occur more commonly as sporadic isolated lesions (70-80% of cases) rather than as multiple lesions in the cerebellum and retina as part of the dominantly inherited familial cancer syndrome, von Hippel–Lindau disease (16-25% of cases). ^[3] Although considered histologically benign, hemangioblastomas may cause significant neurologic deficits, depending on their location. Advances in imaging and microsurgery have markedly improved the treatment of these intraspinal lesions. ^{[4, 5]}

Although hemangioblastoma is a rare tumor of uncertain histotype that typically arises in the cerebellum, quite often in the setting of von Hippel-Lindau disease, Doyle and Fletcher described 22 cases of hemangioblastoma arising at peripheral sites. All the tumors were solitary, except 1, and arose in the spinal nerve roots (12), kidney (3), intestine (2), orbit (1), forearm (1), peritoneum (1), periadrenal soft tissue (1), and flank (1). Five patients had von Hippel-Lindau disease, and another 5 had lesions suggestive of von Hippel-Lindau disease. ^[6]

Magnetic resonance imaging (MRI) of the spine is the diagnostic imaging examination of choice. ^{[7, 8, 9]}  Advances in 3-dimensional digital subtraction angiography (3-D DSA) have made it a useful adjunct in further characterization of the vascular lesion. ^{[10, 11, 12]} Computed tomography (CT) scans of the spine generally demonstrate an isoattenuating, contrast-enhancing lesion. Decreased attenuation, which represents the associated edema, may be present around the tumor. Intraoperative ultrasonography may be a useful adjunct during surgery to help identify the location of the cyst and/or syrinx in relation to the hemangioblastoma.

(See the images below.)

Because spinal hemangioblastomas are relatively rare and share some of the same imaging features with other intramedullary tumors, the diagnosis may be complicated. MRI is particularly helpful in the differential diagnosis. These tumors are almost always associated with a syrinx or significant edema. The edema that accompanies the syrinx varies; some patients have extensive swelling of the spinal cord and only a small neoplasm. Hemangioblastomas and, less often, astrocytomas have the highest proportion of syrinx formation.

Gadolinium-enhanced MRI is the primary diagnostic imaging modality for hemangioblastomas of the spine. The tumors are located mainly on the dorsal aspect of the spinal cord in the cervical and thoracic regions and appear as cystic lesions with mural nodules (see the images below). T1-weighted images show a homogeneously enhancing tumor with a rostral-caudal cyst. Serpentine signal voids on the dorsal surface of the spinal cord may be present.

T2-weighted images more clearly demonstrate the extent of the associated edema and cyst formation (see the following image). Gadolinium-enhanced MRIs may highlight a mural nodule within the cyst wall. ^[13]

Gadolinium-based contrast agents have been linked to the development of nephrogenic systemic fibrosis (NSF) or nephrogenic fibrosing dermopathy (NFD).

Spinal angiography is indicated for large neoplasms when the diagnosis is indeterminate on MRI. ^[14] Angiography reveals a hypervascular, well-delineated tumor mass, as shown in the image below. No arteriovenous shunting occurs in these tumors. Angiograms clearly delineate the feeding and draining vessels; these findings may be helpful for surgical planning and tumor resection. ^{[10, 15, 7]}

Typically, the neoplasms are present on the dorsal surface of the spinal cord. These tumors receive their blood supply from the lateral or posterior spinal arteries. The anterior spinal artery mainly supplies neoplasms that abut the ventral pial surface. In contrast, the venous drainage pattern is unpredictable on MRI. These tumors may drain through the anterior or posterior surface veins and in a rostral or caudal direction. However, more extensive edema appears to be present on T2-weighted MRIs when the drainage predominantly occurs via the anterior surface veins.

One study reported on the use of intraoperative indocyanine green (ICG) videoangiography during surgical resection of spinal cord hemangioblastomas in 7 patients. According to the authors, ICG videoangiography clearly revealed the feeding arteries and enlarged draining veins and assisted in defining the tumor borders in 5 of the 7 patients. One patient had a devascularized residual tumor deep in the spinal cord parenchyma that did not take up the fluorescent dye and therefore was not visualized by ICG videoangiography. In another patient, only the draining veins could be visualized in the ventrolateral tumor because it was covered by the spinal cord parenchyma. ^[16]

Ho VB, Smirniotopoulos JG, Murphy FM, Rushing EJ. Radiologic-pathologic correlation: hemangioblastoma. AJNR Am J Neuroradiol. 1992 Sep-Oct. 13(5):1343-52. [Medline].

Westwick HJ, Giguère JF, Shamji MF. Incidence and Prognosis of Spinal Hemangioblastoma: A Surveillance Epidemiology and End Results Study. Neuroepidemiology. 2016. 46 (1):14-23. [Medline].

Neumann HP, Eggert HR, Weigel K, Friedburg H, Wiestler OD, Schollmeyer P. Hemangioblastomas of the central nervous system. A 10-year study with special reference to von Hippel-Lindau syndrome. J Neurosurg. 1989 Jan. 70(1):24-30. [Medline].

Wang C. Spinal hemangioblastoma: report on 68 cases. Neurol Res. 2008 Jul. 30(6):603-9. [Medline].

Prokopienko M, Kunert P, Podgórska A, Marchel A. Surgical treatment of sporadic and von Hippel-Lindau syndrome-associated intramedullary hemangioblastomas. Neurol Neurochir Pol. 2016. 50 (5):349-55. [Medline].

Doyle LA, Fletcher CD. Peripheral hemangioblastoma: clinicopathologic characterization in a series of 22 cases. Am J Surg Pathol. 2014 Jan. 38(1):119-27. [Medline].

Li X, Wang J, Niu J, Hong J, Feng Y. Diagnosis and microsurgical treatment of spinal hemangioblastoma. Neurol Sci. 2016 Jun. 37 (6):899-906. [Medline].

Merhemic Z, Stosic-Opincal T, Thurnher MM. Neuroimaging of Spinal Tumors. Magn Reson Imaging Clin N Am. 2016 Aug. 24 (3):563-79. [Medline].

Pinter NK, Pfiffner TJ, Mechtler LL. Neuroimaging of spine tumors. Handb Clin Neurol. 2016. 136:689-706. [Medline].

Sciubba DM, Mavinkurve GG, Gailloud P, Garonzik IM, Recinos PF, McGirt MJ. Preoperative imaging of cervical spine hemangioblastomas using three-dimensional fusion digital subtraction angiography. Report of two cases. J Neurosurg Spine. 2006 Jul. 5(1):96-100. [Medline].

Bloomer CW, Ackerman A, Bhatia RG. Imaging for spine tumors and new applications. Top Magn Reson Imaging. 2006 Apr. 17(2):69-87. [Medline].

Arima H, Hasegawa T, Togawa D, Yamato Y, Kobayashi S, Yasuda T, et al. Feasibility of a novel diagnostic chart of intramedullary spinal cord tumors in magnetic resonance imaging. Spinal Cord. 2014 Aug 5. [Medline].

Chen CY, Chen PH, Yao MS, Chu JS, Chan WP. MRI of hemangioblastoma in the conus medullaris. Comput Med Imaging Graph. 2008 Jan. 32(1):78-81. [Medline].

Berenstein A, Lasjaunias P. Surgical Neuroangiography: Endovascular Treatment of Spine and Spinal Cord Lesions. New York, NY: Springer-Verlag; 1994. 1994.

Benedetto N, Aquila F, Vannozzi R. Use of near-infrared indocyanine videoangiography and Flow 800 in the resectioning of a spinal cord haemangioblastoma. Br J Neurosurg. 2013 Dec. 27(6):847-9. [Medline].

Hao S, Li D, Ma G, Yang J, Wang G. Application of intraoperative indocyanine green videoangiography for resection of spinal cord hemangioblastoma: advantages and limitations. J Clin Neurosci. 2013 Sep. 20(9):1269-75. [Medline].

Chanland Roonprapunt, MD, PhD Attending Neurosurgeon, Department of Neurosurgery, St Luke’s-Roosevelt Hospital Center

Disclosure: Nothing to disclose.

V Michelle Silvera, MD 

Disclosure: Nothing to disclose.

George I Jallo, MD Professor of Neurosurgery, Pediatrics, and Oncology, Director, Clinical Pediatric Neurosurgery, Department of Neurosurgery, Johns Hopkins University School of Medicine

George I Jallo, MD is a member of the following medical societies: American Association of Neurological Surgeons, American Medical Association, American Society of Pediatric Neurosurgeons

Disclosure: Nothing to disclose.

Bernard D Coombs, MB, ChB, PhD Consulting Staff, Department of Specialist Rehabilitation Services, Hutt Valley District Health Board, New Zealand

Disclosure: Nothing to disclose.

C Douglas Phillips, MD, FACR Director of Head and Neck Imaging, Division of Neuroradiology, New York-Presbyterian Hospital; Professor of Radiology, Weill Cornell Medical College

C Douglas Phillips, MD, FACR is a member of the following medical societies: American College of Radiology, American Medical Association, American Society of Head and Neck Radiology, American Society of Neuroradiology, Association of University Radiologists, Radiological Society of North America

Disclosure: Nothing to disclose.

James G Smirniotopoulos, MD Chief Editor, MedPix®, Lister Hill National Center for Biomedical Communications, US National Library of Medicine; Professorial Lecturer, Department of Radiology, George Washington University School of Medicine and Health Sciences

James G Smirniotopoulos, MD is a member of the following medical societies: American College of Radiology, American Society of Neuroradiology, Radiological Society of North America

Disclosure: Nothing to disclose.

Chi-Shing Zee, MD Chief of Neuroradiology, Professor, Departments of Radiology and Neurosurgery, Keck School of Medicine of the University of Southern California

Chi-Shing Zee, MD is a member of the following medical societies: American Society of Neuroradiology

Disclosure: Nothing to disclose.

Imaging in Spinal Hemangioblastoma 

Research & References of Imaging in Spinal Hemangioblastoma |A&C Accounting And Tax Services
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