Introduction
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.
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.
Background
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
Related eMedicine topics:
Hemangioblastoma (from Neurosurgery)
Spinal Tumors
Vascular Malformations of the Spinal Cord
Hemangioblastoma, Brain
Neuroblastoma
Pathophysiology
Hemangioblastomas 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.
Frequency
International
Hemangioblastomas account for approximately 3% of all intraspinal tumors.1
Sex
Although 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.
Age
Sporadic 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.
Anatomy
A 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.4 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.
Presentation
From 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 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.5,6
Differential Diagnoses
Astrocytoma, Spine
Ependymoma, Spine
Ganglioglioma
Oligodendroglioma
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References
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Eskridge JM, McAuliffe W, Harris B, Kim DK, Scott J, Winn HR. Preoperative endovascular embolization of craniospinal hemangioblastomas. AJNR Am J Neuroradiol. Mar 1996;17(3):525-31. [Medline]. [Full Text].
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Schwartz TH, Hibshoosh H, Riedel CJ. Estrogen and progesterone receptor-negative T11 vertebral hemangioma presenting as a postpartum compression fracture: case report and management. Neurosurgery. Jan 2000;46(1):218-21. [Medline].
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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. Sep 1976;(3):141-8. [Medline].
Further Reading
Keywords
hemangioblastoma of the spine, capillary hemangioblastoma, capillary hemangioendothelioma, angioreticuloma
