Von Hippel-Lindau disease with spinal hemangioblastoma

Discussion:

Spinal hemangioblastomas are less common than those located in the cerebellar hemispheres (accounting for 80% of such tumors). They are commonly located on the posterior aspect of the spinal cord and 70% are associated with syringomyelia or cystic component. Contrast-enhanced MRI optimizes visualization of the small mural nodules.

The combination of multiple hemangioblastomas, retinal lesions and multiple renal and pancreatic cysts is characteristic of von Hippel Lindau disease

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