= ANGIOBLASTOMA = ANGIORETICULOMA

Prevalence: 1–7.2% of all spinal cord tumors; mostly sporadic

Associated with: Von Hippel-Lindau disease (in ⅓)

Recommendation: screening MR imaging of brain + spine in patients with Von Hippel-Lindau syndrome

Age: middle age; M÷F = 1÷1

Path: nonglial highly vascular discrete nodular masses abutting leptomeninges with prominent dilated + tortuous vessels on posterior cord surface

Histo: large pale stromal cells of unknown origin packed between blood vessels of varying sizes

Location: intramedullary (75%), radicular (20%), intradural extramedullary (5%); thoracic cord (50%), cervical cord (40%); solitary in >80%, multiple lesions indicate Von Hippel-Lindau syndrome + require screening of entire spine

Site: subpial aspect of dorsal spine; may extend exophytically into subarachnoid / extradural space

• mean duration of symptoms is 38 months:
  • sensory changes (39%): impaired proprioception
  • motor dysfunction (31%), pain (31%)
• increased interpediculate distance (mass effect)

Angio:

• highly vascular mass with dense prolonged blush
• large draining veins form sinuous mass along posterior aspect of cord

MR:

• iso- (50%) / hyperintense (25%) diffuse cord expansion on T1WI
• hyperintense lesion with intermixed focal flow voids on T2WI:
  • curvilinear areas of signal voids
• cyst formation / syringohydromyelia (in up to 100%):
  • intratumoral cystic component (50–60%)
  • occasionally cystic mass with enhancing mural nodule (CLASSIC for cerebellar hemangioblastoma)
  • densely staining tumor nodule
• ± surrounding edema and “cap” sign
• well-demarcated Gd-enhancing mass

Cx: intramedullary hemorrhage, hematomyelia, subarachnoid hemorrhage (rare)

DDx: arteriovenous fistula (not well circumscribed, heterogeneous signal intensity)