Cavernous Hemangioma

Cavernous Hemangioma
Brock Kaya, MD
Key Facts
Terminology
  • Angiographically occult vascular malformation composed of uniform blood vessels with fibrous walls
Clinical Issues
  • Account for 10-15% of all vascular malformations
  • Majority (85%) are supratentorial
  • In children, most common manifestation is hemorrhage
Image Findings
  • “Popcorn ball” appearance with mixed hyperintense and hypointense signal intensity on both T1- and T2-weighted sequences
  • Typical low signal intensity ring surrounding lesion due to hemosiderin
Macroscopic Features
  • Usually small, red-purple, lobulated, mulberry-like lesions
  • Range in size: Millimeters to several centimeters (diameter)
Microscopic Pathology
  • Circumscribed, compact aggregate of dilated, thick-walled blood vessels with hyalinized walls
  • Vascular walls lack smooth muscle and elastic lamina
  • Little to no intervening brain parenchyma present between blood vessels
  • Hemosiderin deposition and gliosis in surrounding brain parenchyma
Graphic depicts a cavernous hemangioma of the pons, consisting of a compact aggregate of dilated blood vessels with surrounding hemosiderin deposits.
Axial T1WI MR demonstrates a left temporal cavernous hemangioma with central bright T1 contents. A cavernoma image was previously resected on the right. (Courtesy S. M. Holmes, MD.)
TERMINOLOGY
Synonyms
  • Cavernous angioma, cavernous malformation, cavernoma
Definitions
  • Angiographically occult vascular malformation composed of uniform blood vessels with fibrous walls
ETIOLOGY/PATHOGENESIS
Acquired Anomaly
  • Previously considered congenital, but now favored to be acquired
  • Familial forms
    • Autosomal dominant with incomplete penetrance
    • Related to mutations in either KRIT1 (CCM1), CCM2, or PDCD10 (CCM3)
  • May occur secondarily following radiation therapy for childhood brain tumors
CLINICAL ISSUES
Epidemiology
  • Incidence: 0.5-0.7%
  • Account for 10-15% of all vascular malformations
  • Equal sex distribution
  • Between 9-25% of cases occur in children
  • Sporadic and familial forms
    • Sporadic lesions typically solitary
    • Multiple cavernomas either familial or sporadic
  • May have associated venous angioma (developmental venous anomaly)
Site
  • Found throughout CNS
  • Majority (85%) are supratentorial
  • Subcortical white matter is most common site
  • Pons and cerebellum are common infratentorial sites
Presentation
  • Increasingly, an incidental finding by imaging
  • Most are asymptomatic
  • Symptomatic individuals (25% of those affected) may present at any age but most common in 2nd to 5th decades
    • Seizures
    • Focal neurologic deficits
    • Hemorrhage
      • In adults, uncommon cause of clinically significant cerebral hemorrhage (risk of 0.25-6% per year)
      • In children, most common manifestation is hemorrhage
Treatment
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Jul 8, 2016 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Cavernous Hemangioma

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