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Cerebral Cavernous Malformation (Cavernoma)

Last Updated: October 1, 2018

Open Table of Contents: Cerebral Cavernous Malformation (Cavernoma)

Figure 1: MR images demonstrate a large parenchymal lesion centered in the right cerebellar hemisphere causing significant adjacent mass effect and complete obstruction of the fourth ventricle. There is central reduced diffusivity (top row) and a large amount of susceptibility (blooming) artifact on SWI (middle row left) secondary to recent hemorrhage. The FLAIR image (middle row right) demonstrates a moderate amount of hyperintense signal which is typically only seen in lesions that have recently hemorrhaged or occasionally when the lesions are large and have a significant amount of mass effect. Somewhat atypical is the degree of heterogeneous enhancement on post-contrast image (bottom row).

Figure 1: MR images demonstrate a large parenchymal lesion centered in the right cerebellar hemisphere causing significant adjacent mass effect and complete obstruction of the fourth ventricle. There is central reduced diffusivity (top row) and a large amount of susceptibility (blooming) artifact on SWI (middle row left) secondary to recent hemorrhage. The FLAIR image (middle row right) demonstrates a moderate amount of hyperintense signal which is typically only seen in lesions that have recently hemorrhaged or occasionally when the lesions are large and have a significant amount of mass effect. Somewhat atypical is the degree of heterogeneous enhancement on post-contrast image (bottom row).

Description

  • Benign vascular lesion with a classic imaging appearance

Pathology

  • Blood cavities surrounded by a single layer of endothelium without muscular tissue or intervening brain parenchyma
  • Two types:
    • Sporadic and familial

Clinical Features

  • Symptoms
    • Seizure, hemorrhage, focal progressive neurologic deficits, and headaches
    • ~25% are asymptomatic
  • Age
    • Peak presentation in middle age
    • Familial cavernous malformations tend to present earlier
  • Gender
    • No gender predilection
  • Associations
    • Developmental venous anomaly (DVA)
    • Superficial siderosis (hemosiderin deposition on the surface of the brain)
    • Cutaneous abnormalities
      • Café au lait spots
      • Hyperkeratotic capillary-venous malformation

Imaging

  • General
    • Classic “popcorn ball” appearance with complete hypointense rim on T2
  • Modality specific
    • CT
      • Often negative
      • If positive, will see a well-defined hyperdense lesion with associated calcifications that demonstrates little to no enhancement
    • MR
      • T1WI and T2WI
        • “popcorn ball” appearance with low signal rim and mixed internal signal demonstrating differing stages of hemorrhage
        • Surrounding edema only present if recent hemorrhage has occurred
      • T2*
        • Hypointense signal associated due to hemosiderin and calcification
      • Contrast
        • Minimal or no contrast enhancement, unless associated with a developmental venous anomaly
    • Conventional Angiogram
      • Usually normal
      • Occasional slow intralesional flow without AV shunting and venous pooling
  • Imaging Recommendations
    • MR with contrast
    • Contrast used to exclude associated anomalies
    • T2*/SWI/GRE sequence with long TE
  • Mimic
    • When recently hemorrhagic, it can be difficult to distinguish from an underlying hemorrhagic neoplasm or an AVM. Followup imaging is often a necessary approach to monitor the evolution of the blood products and to evaluate for an underlying neoplasm.

For more information, please see the corresponding chapter in Radiopaedia, and the Cavernous Malformation chapter within the Cerebral Vascular Diseases sub-volume within the Neurosurgical Atlas.

Contributor: Sean Dodson, MD

DOI: https://doi.org/10.18791/nsatlas.v1.03.02.09

References

Brunereau L, et al. Familial Form of Intracranial Cavernous Angioma: MR Imaging Findings in 51 Families. Radiology. 2000; 214(1):209-16.

Husiman T. Tumor-like lesions of the brain. Cancer Imaging. 2009; 9, S10-S13.

Meng G, et al. The association between cerebral developmental venous anomaly and concomitant cavernous malformation: an observational study using magnetic resonance imaging. BMC Neurol. 2014; 14:50.

Moore SA, et al. Long-term natural history of incidentally discovered cavernous malformations in a single-center cohort. J Neurosurg. 2014; 120(5):1188-92.

Sohn CH, et al. Characteristic MR Imaging Findings of Cavernous Hemangiomas in the Cavernous Sinus. AJNR. 2003; 24:1148-51.

Tamburrini G, et al. Large cerebral cavernoma mimicking a brain tumor. Pediatr Neurosurg. 2002; 37(2):105-6.

Yun TJ, et al. A T1 Hyperintense Perilesional Signal Aids in the Differentiation of a Cavernous Angioma from Other Hemorrhagic Masses. AJNR. 2008; 29:494-500.

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