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Cavernous Malformation

Last Updated: January 10, 2022

Open Table of Contents: Cavernous Malformation

Figure 1: Axial T1-, T2-, and susceptibility-weighted images reveal a "popcorn" lesion within the left postcentral gyrus demonstrating heterogenous T1 and T2 signals accompanied by a hypointense rim of blooming artifact (hemosiderin) typical of a cavernous malformation.

Figure 1: Axial T1-, T2-, and susceptibility-weighted images reveal a "popcorn" lesion within the left postcentral gyrus demonstrating heterogenous T1 and T2 signals accompanied by a hypointense rim of blooming artifact (hemosiderin) typical of a cavernous malformation.

Overview

Cavernous malformations are benign vascular hamartomas consisting of dysplastic immature vascular channels with variable mass effect depending on size. Although nearly always angiographically occult, these lesions may be dynamic in their behavior over time, with evidence of hemorrhagic products of mixed stages, and may present with acute parenchymal hemorrhage. Presentation can include seizure (up to 50%) or focal neurologic deficit (25%) or can be asymptomatic/incidental (20%).

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Imaging

  • General characteristics
    • Masses of immature, cavern-like vascular channels or blood pools of variable complexity
      • Loculated intralesional hemorrhages with different stages of blood product evolution
      • Complete hemosiderin rim usually surrounds lesion
      • Lesions classically described as having a "popcorn" appearance on MRI
      • Dynamic behavior on serial imaging, including enlargement, regression, and de novo formation
    • Locations
      • Occurs throughout the central nervous system
      • Brain parenchyma (common)
      • Spinal cord (rare, more common in patients with multiple cavernous malformation syndrome)
      • Extraaxial (rare)
      • Can originate within any venous sinus (cavernous sinus is the most common site)
    • Size
      • Can attain large size before becoming symptomatic
      • Varies from microscopic to giant (>6 cm)
      • Majority fall in the range of 0.5 to 4.0 cm
    • Multiple cavernous malformations
      • Multiple (familial) cavernous malformation syndrome
        • Autosomal dominant, variable penetrance
        • Nonsense, frame-shift, or splice-site mutations consistent with 2-hit model
      • Radiation induced
    • Associated abnormalities
      • Developmental venous anomaly
      • Superficial siderosis
      • Cutaneous abnormalities
      • Café au lait spots
      • Hyperkeratotic capillary-venous malformations
    • Differential diagnoses
      • Arteriovenous malformation
      • Hemorrhagic neoplasm
      • Calcified neoplasm
      • Hypertensive microbleeds
      • Amyloid angiopathy
  • Nonenhanced CT
    • Technique
      • Axial 5-mm collimation is typical
      • Reformatted images in coronal/sagittal planes can be made available for difficult cases, although they are generally not needed
    • Findings
      • Negative in up to 50% of cases without recent parenchymal hemorrhage
      • Well-delineated round/ovoid hyperdense lesion, usually <3 cm
      • Partially calcified in some cases
      • No evidence of surrounding hypodense edema or mass effect (unless recent hemorrhage)
  • CT angiography
    • Findings
      • Minimal to no enhancement, usually negative (angiographically occult)
  • MRI
    • Technique
      • Multiple pulse sequences available to evaluate various tissue properties as well as dynamic properties (flow, velocity)
    • Findings
      • Most commonly identified as a popcorn-like mass lesion with mixed signal on T1- and T2-weighted and FLAIR imaging, reflecting mixed-blood–containing loculations
      • T1 signal
        • Variable depending on blood product evolution
      • T2 signal
        • Variable depending on blood product evolution
        • T2-hypointense hemosiderin rim
        • May see loculations of blood with fluid-fluid levels
      • FLAIR
        • Again, variable signal dependent on age of blood products
        • May reveal perilesional parenchymal edema if recently hemorrhagic
      • T2* GRE, SWI
        • Prominent hypointense blooming from susceptibility artifact
        • Very sensitive for detection, particularly in cases of multiple lesions, familial syndrome
      • DWI
        • Usually normal
      • T1WI C+
        • Minimal or no enhancement (may show associated developmental venous anomaly)
      • MRA
        • Usually normal (angiographically occult)
    • Pitfalls
      • Large acute hemorrhage may obscure more typical features
  • Digital subtraction angiography
    • Findings
      • Usually normal (angiographically occult vascular malformation)
      • Extradural lesions can be hypervascular
      • Slow intralesional flow without arteriovenous shunting may be seen
      • Avascular mass effect if large or acute hemorrhage
      • Associated other malformations, most commonly developmental venous anomaly
      • In general, there is no current role for endovascular therapy
  • Staging, grading, and classification
    • Zabramski classification: associated with risk of rehemorrhage
      • Type 1 = subacute hemorrhage (hyperintense on T1WI; hyperintense or hypointense on T2WI)
      • Highest risk of rehemorrhage
      • Type 2 = mixed signal intensity on T1WI, T2WI with degrading hemorrhage of various ages
      • Type 3 = chronic hemorrhage (hypointense to isointense on T1WI, T2WI)
      • Type 4 = punctate microhemorrhages (T2*GRE and SWI sequences most sensitive)

For more information, please see the corresponding chapter in Radiopaedia and the Cavernous Malformation chapter within the Brain Tumor Mimics subvolume of The Neurosurgical Atlas.

Contributor: Daniel Murph, MD

DOI: https://doi.org/10.18791/nsatlas.v2.03.03.04.04

References

Chen L, Tanriover G, Yano H, et al. Apoptotic functions of PDCD10/CCM3, the gene mutated in cerebral cavernous malformation 3. Stroke 2009;40:1474–1481. doi.org/10.1161/STROKEAHA.108.527135

Dashti SR, Fiorella D, Spetzler RF, et al. Preoperative Onyx embolization of a giant cavernous malformation involving the dural sinuses. J Neurosurg Pediatr 2009;3:302–306. doi.org/10.3171/2009.1.PEDS08412

Al-Shahi Salman R, Berg MJ, Morrison L, et al. Hemorrhage from cavernous malformations of the brain: definition and reporting standards. Angioma Alliance Scientific Advisory Board. Stroke 2008;39:3222–3230. doi.org/10.1161/STROKEAHA.108.515544

Shenkar R, Venkatasubramanian PN, Zhao JC, et al. Advanced magnetic resonance imaging of cerebral cavernous malformations: part I. High-field imaging of excised human lesions. Neurosurgery 2008;63:782–789. doi.org/10.1227/01.NEU.0000325490.80694.A2

Shenkar R, Venkatasubramanian PN, Zhao JC, et al. Advanced magnetic resonance imaging of cerebral cavernous malformations: part II. Imaging of lesions in murine models. Neurosurgery 2008;63:790–797; discussion 797–798. doi.org/10.1227/01.NEU.0000315862.24920.49

Son DW, Lee SW, Choi CH. Giant cavernous malformation: a case report and review of the literature. J Korean Neurosurg Soc 2008;43:198–200. doi.org/10.3340/jkns.2008.43.4.198

Yun TJ, Na DG, Kwon BJ, et al. A T1 hyperintense perilesional signal aids in the differentiation of a cavernous angioma from other hemorrhagic masses. AJNR Am J Neuroradiol 2008;29:494–500. doi.org/10.3174/ajnr.A0847

Al-Shahi R, Bhattacharya JJ, Currie DG, et al. Prospective, population-based detection of intracranial vascular malformations in adults: the Scottish Intracranial Vascular Malformation Study (SIVMS). Stroke 2003;34:1163–1169. doi.org/10.1161/01.STR.0000069018.90456.C9

Reich P, Winkler J, Straube A, et al. Molecular genetic investigations in the CCM1 gene in sporadic cerebral cavernomas. Neurology 2003;60:1135–1138. doi.org/10.1212/01.wnl.0000055470.62265.44

Rivera PP, Willinsky RA, Porter PJ. Intracranial cavernous malformations. Neuroimaging Clin N Am 2003;13:27–40. doi.org/10.1016/s1052-5149(02)00060-6

Wang CC, Liu A, Zhang JT, et al. Surgical management of brain-stem cavernous malformations: report of 137 cases. Surg Neurol 2003;59:444–454; discussion 454. doi.org/10.1016/s0090-3019(03)00187-3

Biondi A, Clemenceau S, Dormont D, et al. Intracranial extra-axial cavernous (HEM) angiomas: tumors or vascular malformations? J Neuroradiol 2002;29:91–104.

Kehrer-Sawatzki H, Wilda M, Braun VM, et al. Mutation and expression analysis of the KRIT1 gene associated with cerebral cavernous malformations (CCM1). Acta Neuropathol (Berl) 2002;104:231–240. doi.org/10.1007/s00401-002-0552-6

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