Spinal Cord Hemangioblastoma, Von Hippel Lindau Syndrome
Findings:
MR images of the lumbar spine without and with contrast demonstrate a large intensely enhancing mass contiguous with the distal spinal cord at the L1 level. Numerous tortuous vessels extend superiorly from the mass. The mass occupies the spinal canal and appears intramedullary. Localizer images for the MR demonstrate extensive cystic changes of the pancreas.
Differential Diagnosis:
Hemangioblastoma, Ependymoma, Astrocytoma, Metastasis, AVM. The presence of marked hypervascularity and numerous feeding vessels without a discrete nidus makes hemangioblastoma (HBL) most likely, as well as the other features of VHL.
Discussion:
A discussion of Von Hippel Lindau Syndrome is found elsewhere on this site, under hemangioblastoma. An important point is that isolated HBL is more commonly sporadic than related to VHL, and multiple HBL are nearly exclusively seen in VHL.