Cavernous Hemangioma



Cavernous Hemangioma


Brock Kaya, MD









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



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

Jul 8, 2016 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Cavernous Hemangioma
Premium Wordpress Themes by UFO Themes
%d bloggers like this: