Imaging in Spine Hemangioblastoma
- Author: Chanland Roonprapunt, MD, PhD; Chief Editor: James G Smirniotopoulos, MD more...
Overview
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] 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).[2] Although considered histologically benign, hemangioblastomas may cause significant neurologic deficits, depending on their location. Recent advances in imaging and microsurgery have markedly improved the treatment of these intraspinal lesions.[3]
Preferred examination
Magnetic resonance imaging (MRI) of the spine is the diagnostic imaging examination of choice. Recent advances in three-dimensional digital subtraction angiography (3-D DSA) have made it a useful adjunct in further characterization of the vascular lesion.[4, 5] See the images below.
Sagittal T1-weighted image demonstrates the dorsal location of the tumor, which enhances homogeneously. This tumor does not show any flow voids that may be seen with larger tumors. Note that the spinal cord rostral and caudal to the tumor is quite swollen from edema. 
Spinal angiogram demonstrates a large cervical hemangioblastoma. Tumor blush is quite evident on this selective injection. The tumor receives a vascular supply from both the anterior and posterior spinal arteries. Embolization is not feasible for these tumors because of the small caliber of the feeding vessels. However, the angiogram is important to define the vascular anatomy for large tumors. Computed Tomography
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.
Magnetic Resonance Imaging
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.
Sagittal T1-weighted image demonstrates the dorsal location of the tumor, which enhances homogeneously. This tumor does not show any flow voids that may be seen with larger tumors. Note that the spinal cord rostral and caudal to the tumor is quite swollen from edema. 
Axial T1-weighted image confirms the dorsal hemangioblastoma that abuts the pial surface, whose position is typical of these tumors and makes removal easy. 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.[6]
T2-weighted image demonstrates the extensive edema typically associated with hemangioblastomas of the spine. This edema extends rostrally to the medulla and caudally to the upper thoracic spinal cord. If the tumor is left untreated, the edema develops into a syrinx. Gadolinium-based contrast agents (gadopentetate dimeglumine [Magnevist], gadobenate dimeglumine [MultiHance], gadodiamide [Omniscan], gadoversetamide [OptiMARK], gadoteridol [ProHance]) have been linked to the development of nephrogenic systemic fibrosis (NSF) or nephrogenic fibrosing dermopathy (NFD). For more information, see the eMedicine topic Nephrogenic Fibrosing Dermopathy. The disease has occurred in patients with moderate to end-stage renal disease after being given a gadolinium-based contrast agent to enhance MRI or MRA scans.
NSF/NFD is a debilitating and sometimes fatal disease. Characteristics include red or dark patches on the skin; burning, itching, swelling, hardening, and tightening of the skin; yellow spots on the whites of the eyes; joint stiffness with trouble moving or straightening the arms, hands, legs, or feet; pain deep in the hip bones or ribs; and muscle weakness. For more information, see FDA Information on Gadolinium-Based Contrast Agents or Medscape.
Differential diagnosis
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.
Ultrasonography
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.
Angiography
Spinal angiography is indicated for large neoplasms when the diagnosis is indeterminate on MRI.[7] 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.[4]
Spinal angiogram demonstrates a large cervical hemangioblastoma. Tumor blush is quite evident on this selective injection. The tumor receives a vascular supply from both the anterior and posterior spinal arteries. Embolization is not feasible for these tumors because of the small caliber of the feeding vessels. However, the angiogram is important to define the vascular anatomy for large tumors. 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.
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