You are in: eMedicine Specialties > Radiology > BRAIN/SPINE Hemangioblastoma, SpineArticle Last Updated: Mar 21, 2007AUTHOR AND EDITOR INFORMATIONSection 1 of 11
  Author: Chanland Roonprapunt, MD, PhD, Staff Physician, Department of Neurosurgery, Chiari Institute, North Shore University Coauthor(s): V Michelle Silvera, MD, Division of Pediatric Neuroradiology, Clinical Instructor, Boston Children's Hospital; George Jallo, MD, Associate Professor of Neurosurgery, Pediatrics and Oncology, Department of Neurosurgery, Division of Pediatric Neurosurgery, Johns Hopkins University School of Medicine Editors: Chi-Shing Zee, MD, Chief of Neuroradiology, Professor, Departments of Radiology and Neurosurgery, University of Southern California School of Medicine; Bernard D Coombs, MB, ChB, PhD, Consulting Staff, Department of Specialist Rehabilitation Services, Hutt Valley District Health Board, New Zealand; C Douglas Phillips, MD, Professor, Departments of Radiology, Neurosurgery, and Otolaryngology, University of Virginia Health Sciences Center; Robert M Krasny, MD, Consulting Staff, Department of Radiology, The Angeles Clinic and Research Institute; James G Smirniotopoulos, MD, Professor of Radiology, Neurology, and Biomedical Informatics, Chairman, Department of Radiology and Radiological Sciences, Uniformed Services University of the Health Sciences Author and Editor Disclosure Synonyms and related keywords: capillary hemangioblastoma, capillary hemangioendothelioma, angioreticuloma INTRODUCTIONSection 2 of 11
  BackgroundHemangioblastomas 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. PathophysiologyHemangioblastomas are composed of neoplastic stromal cells surrounded by an abundant blood-filled endothelial network. On gross examination, hemangioblastomas are well-circumscribed tumors that may be either solid or cystic with a mural nodule. Surrounding edema is common but variable; some patients may have extensive swelling of the spinal cord and only a small neoplasm. All patients who have hemangioblastomas in the spinal cord should undergo a complete clinical assessment for evidence of von Hippel–Lindau disease, a familial neoplastic disorder that is characterized by hemangioblastomas and endolymphatic sac cystadenomas within the central nervous system (CNS), abdominal pancreatic and renal cysts, pancreatic adenomas, islet cell tumors, and renal cell carcinoma. Determination of the relevant family history and neurologic and ophthalmologic examinations with complete neuraxis and abdominal imaging studies are warranted. FrequencyInternationalHemangioblastomas account for approximately 3% of all intraspinal tumors.1 SexAlthough hemangioblastomas can occur in pregnant women, they do not seem to be associated with female hormonal activity. Indeed, an overall 2-to-1 male preponderance exists. Hemangioblastomas may occur more often during pregnancy because of the associated hemodynamic changes rather than the hormonal changes during pregnancy, as reported with vertebral hemangioblastoma. AgeSporadic cases of hemangioblastomas typically occur in persons aged 30-40 years, with a mean patient age of approximately 36 years. Cases associated with von Hippel–Lindau disease usually occur in those aged 20-30 years, with multiple lesions. AnatomyA review of the literature shows that most (85%) isolated hemangioblastomas arise in the cervical and thoracic portions of the spinal cord, mainly on the dorsal aspect.3 Cysts, syringes, or both may be present rostral or caudal to the tumor. The posterior or lateral feeding arteries are the most common feeding vessels, and the anterior spinal artery is involved in one third of cases. The drainage pattern through the anterior and posterior surface veins is variable and not specific. Clinical DetailsFrom the time of the initial diagnosis, hemangioblastomas have a slow, indolent course. The formation of edema and cysts and/or syringes can exacerbate the symptoms. Similar to that of other intramedullary neoplasms, the most common complaint of patients with hemangioblastomas is pain. Sensory changes, motor deficits, scoliosis, and bowel and/or bladder abnormalities are other presenting problems; these symptoms are related to the location of the tumor. Preferred ExaminationMagnetic 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. DIFFERENTIALSSection 3 of 11
Astrocytoma, Spine Ependymoma, Spine Ganglioglioma Oligodendroglioma RADIOGRAPHSection 4 of 11
FindingsPlain radiographs of the spine do not provide any diagnostic information regarding this soft-tissue tumor. CT SCANSection 5 of 11
FindingsComputed tomography (CT) scans of the spine generally demonstrate an iso-attenuating, contrast-enhancing lesion. Decreased attenuation, which represents the associated edema, may be present around the tumor. MRISection 6 of 11
FindingsGadolinium-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. 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. Gadolinium-enhanced MRIs may highlight a mural nodule within the cyst wall. 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-based contrast agents (gadopentetate dimeglumine [Magnevist], gadobenate dimeglumine [MultiHance], gadodiamide [Omniscan], gadoversetamide [OptiMARK], gadoteridol [ProHance]) have recently 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. As of late December 2006, the FDA had received reports of 90 such cases. Worldwide, over 200 cases have been reported, according to the FDA. 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 movingor straightening the arms, hands, legs, or feet; pain deep in the hip bones or ribs; and muscle weakness. For more information, see the FDA Public Health Advisory or Medscape. ULTRASOUNDSection 7 of 11
FindingsIntraoperative ultrasonography may be a useful adjunct during surgery to help identify the location of the cyst and/or syrinx in relation to the hemangioblastoma. ANGIOGRAPHYSection 8 of 11
FindingsSpinal angiography is indicated for large neoplasms when the diagnosis is indeterminate on MRI. Angiography reveals a hypervascular, well-delineated tumor mass. 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. 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. INTERVENTIONSection 9 of 11
Complete microsurgical removal remains the treatment of choice for isolated symptomatic spinal hemangioblastomas. Preoperative embolization has been used as an adjunct to surgery, with only modest benefits. Superselective embolization of the feeding vessels may be difficult and fraught with complications. Embolization without superselective microcatheter positioning in the tumor may compromise the normal spinal cord supply and injure viable spinal parenchyma. MULTIMEDIASection 10 of 11
REFERENCESSection 11 of 11
Hemangioblastoma, Spine excerpt Article Last Updated: Mar 21, 2007 | |||||||||||||||||||||||||||||||||||
