J Korean Soc Radiol.  2015 Mar;72(3):171-175. 10.3348/jksr.2015.72.3.171.

Multifocal Spinal Hemangioblastoma in von Hippel-Lindau Syndrome: A Case Report and Literature Review

• Kim OH

• KMID: 2161168
• DOI: http://doi.org/10.3348/jksr.2015.72.3.171

Abstract

Hemangioblastoma is a benign vascular neoplasm of the central nervous system that occurs frequently in the cerebellum and other areas of the central nervous system including spinal cord and brainstem. Spinal hemangioblastoma can present as a sporadic isolated lesion or as a component of von Hippel-Lindau syndrome. The author presents a case of 32-year-old man with von Hippel-Lindau syndrome and spinal hemangioblastomas represented by multiple small spinal lesions, with an emphasis on the magnetic resonance imaging findings and clinical characteristics of von Hippel-Lindau syndrome-associated spinal hemangioblastomas.

MeSH Terms

Adult
Brain Stem
Central Nervous System
Cerebellum
Hemangioblastoma*
Humans
Magnetic Resonance Imaging
Spinal Cord
Spinal Cord Neoplasms
Vascular Neoplasms
von Hippel-Lindau Disease*

Figure

• 

  Fig. 1 A 32-year-old man with von Hippel-Lindau syndrome. A. Brain MRI shows an intra-axial cystic mass with an intensely enhancing mural nodule (arrow) in the right cerebellar hemisphere, representing a cerebellar hemangioblastoma. B-E. Sagittal fat suppressed T1WI after gadolinium administration of cervical (B), thoracic (C) and lumbar (D) spines demonstrate the multiple, scattered, tiny and small, intensely gadolinium-enhancing masses, located in the dorsal aspects of the spinal cord including the cauda equina (arrows), and the prominent wavy vessels adjacent to a thoracic spinal mass (white arrowheads) at the level of T8. Sagittal T2WI (E) of the thoracic spine demonstrates a small, intramedullary, isointense mass in the dorsal aspect of the spinal cord with the peritumoral edematous signal changes (black arrowheads) at the T8 level. F. Axial portal-phase abdominal CT after intravenous contrast administration shows the multiple, lobular, septated, cystic masses with tiny septal calcifications in the pancreatic tail (arrow), body, and head (not shown). These CT findings are compatible with serous cystadenoma of the pancreas. A hepatic cyst in the right lobe (thin arrow). G-K. At the 1-year follow-up, sagittal T1WI (G), serial sagittal T2WI (H, I) and series sagittal fat supressed T1WI after gadolinium administration (J, K) demonstrate the newly developed small syrinx showing the fluid-like signal intensity (open arrows) and more prominent peritumoral edema (black arrowheads) adjancet to the spinal mass (arrows) at the level of T8. Note.-T1WI = T1-weighted imaging, T2WI = T2-weighted imaging

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