Unilateral cerebellar hypoplasia
Heather Borders, MD
over 7 years ago
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Hemangioblastoma - Spinal Cord
Joseph Junewick, MD FACR
|Diagnostic Category: Neoplasia Malignant
|Created: over 5 years ago
|Updated: over 5 years ago
Teenager with vonHippel-Lindau disease with new painful scoliosis.
MR – Sagittal T2 and postgadolinium T1 images of the upper spine show enhancing intramedullary nodules at C3 and C5-CC6 with associated cysts and edema.
CNS hemangioblastoma is one of the most common manifestations of VHL disease. The cerebellum and spinal cord are the most common sites with the medulla and supratentorial region being uncommon. Only 5%–30% of all cerebellar hemangioblastomas are attributed to VHL disease, whereas 80% of spinal cord hemangioblastomas occur with the disease. Although most of these tumors (75%) are intramedullary, they may involve the dura or even be extradural. Within the spinal cord, the thoracic region is most commonly involved followed closely by the cervical cord. Most cord hemangioblastomas are solitary and slow growing. Hemangioblastomas manifest with diffuse cord expansion and variable signal intensity on T1-weighted images, with the most common appearance being isointense or hyperintense relative to the normal spinal cord. On T2-weighted images, these lesions characteristically have high signal intensity with intermixed focal flow voids. Surrounding edema and a cap sign may also be seen. Tumoral cyst and/or syringohydromyelia is very common. Focal spinal pain is the most common symptom of spinal cord hemangioblastoma, however signs of obstructive hydrocephalus and spinal cord compression may also be encountered. Treatment of CNS hemangioblastomas involves surgical resection of symptomatic tumors, with preoperative arterial embolization for extensive spinal cord tumors.
Leung RS, Biswas SV, Duncan M, et al. Imaging Features of von Hippel–Lindau. RadioGraphics (2008); 28:65-79.
Koeller KK, Rosenblum RS, Morrison AL. Neoplasms of the Spinal Cord and Filum Terminale: Radiologic-Pathologic Correlation. RadioGraphics (2000); 20:1721-1749.