Cavernous Malformation Classification

In its sporadic form, CA occurs as a solitary hemorrhagic vascular lesion or as clustered lesions associated with a developmental venous anomaly. In its autosomal dominant familial form (Online Mendelian Inheritance in Man #116860), CA is caused by a heterozygous germline loss-of-function mutation in one of three genes—CCM1/KRIT1, CCM2/Malcavernin, and CCM3/PDCD10—causing multifocal lesions throughout the brain and spinal cord.

see Intracranial cavernous malformation

see Intradiploic cavernous hemangioma

see Spinal epidural cavernous hemangioma

see Spinal cord cavernoma…

Cavernous malformations (cavernous angiomas, cavernomas, cavernous hemangiomas) are well-defined, grossly visible lesions that may reach a significant size. They are composed of a compact mass of sinusoidal-type vessels immediately in apposition to each other without any recognizable intervening neural parenchyma.

Estimated prevalence between 0·4 and 0·9% ¹⁾, appearing mainly as singular supratentorial lesions ²⁾.

Their biology is usually benign without changes in size, although the potential for growth and recurrent bleeding is well documented ³⁾ ⁴⁾ ⁵⁾ ⁶⁾ ⁷⁾.

¹⁾

Bertalanffy H, Benes L, Miyazawa T, Alberti O, Siegel AM, Sure U. Cerebral cavernomas in the adult. Review of the literature and analysis of 72 surgically treated patients. Neurosurg Rev. 2002;25:1–53.

²⁾

Cappabianca P, Alfieri A, Maiuri F, Mariniello G, Cirillo S, de Divitiis E. Supratentorial cavernous malformations and epilepsy: seizure outcome after lesionectomy on a series of 35 patients. Clin Neurol Neurosurg. 1997;99:179–83.

³⁾

Dalyai RT, Ghobrial G, Awad I, Tjoumakaris S, Gonzalez LF, Dumont AS, et al. Management of incidental cavernous malformations: a review. Neurosurg Focus. 2011;31:E5.

⁴⁾

Kivelev J, Niemelä M, Hernesniemi J. Characteristics of cavernomas of the brain and spine. J Clin Neurosci. 2012;19:643–48.

⁵⁾

Pozzati E, Acciarri N, Tognetti F, Marliani F, Giangaspero F. Growth, subsequent bleeding, and de novo appearance of cerebral cavernous angiomas. Neurosurgery. 1996;38:662–9.

⁶⁾

Maiuri F, Cappabianca P, Gangemi M, De Caro Mdel B, Esposito F, Pettinato G, et al. Clinical progression and familial occurence of cerebral cavernous angiomas: the role of angiogenic and growth factors. Neurosurg Focus. 2006;21:e3.

⁷⁾

Gross BA, Lin N, Du R, Day AL. The natural history of intracranial cavernous malformations. Neurosurg Focus. 2011;30:E24.