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cavernous_malformation

Cavernous malformation

General information

Key concepts

usually angiographically occult. May show up on MRI (open channels → flow void on T2WI, previous hemorrhage →“popcorn” pattern especially on T2* gradient echo) or contrast CT

● low-flow. No intervening neural parenchyma, no arteries. Associated with venous anomaly (represents venous outflow and should be preserved)

XRT is a risk factor for developing cavernous malformation

● presentation: usually seizures:

The 5-year risk of first-time seizure was 6% among CM patients presenting with symptoms vs. 4% for those with incidental CMs

● Hemorrhage: rare, the risk is difficult to predict (see Cavernous malformation hemorrhage)

● treatment:

a) surgery is the treatment of choice for symptomatic accessible lesions

b) radiosurgery should not be considered as a treatment option


Cavernous angioma (CA) is also known as cavernoma, cavernous hemangioma, and cerebral cavernous malformation (CCM) (National Library of Medicine Medical Subject heading unique ID D006392).

It is classified as a group of vascular malformations, where a collection of dilated blood vessels form a tumor.

Hubert von Luschka was the first to describe a cerebral (Cavernöse Blutgeschwulst) (cavernous malformation) in 1854.

Synonyms

The terms cavernoma, cavernous hemangioma, cavernous angioma, and cavernous malformation are used interchangeably and appear equally frequent in the literature. The terms cavernoma, cavernous angioma, and cavernous hemangioma suggest that it is a neoplastic lesion, which is inaccurate as cavernous malformations are true vascular malformations.

The term cavernous hemangioma is also confusing as cavernous hemangiomas in the cavernous sinus or in the orbit; both are slow-growing neoplastic diseases unrelated to vascular 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.

In 1928, Cushing and Bailey classified cavernous malformations as a solid subtype of hemangioblastomas 1)

see Intradiploic cavernous hemangioma

see Intracranial cavernous malformation

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% 2), appearing mainly as singular supratentorial lesions 3).

Their biology is usually benign without changes in size, although the potential for growth and recurrent bleeding is well documented 4) 5) 6) 7) 8).

Etiology

see Cavernous malformation etiology.

Pathology

Cavernous malformation (CCM)s are benign, low-flow vascular lesions with thin elastic endothelial walls that lack adventitial smooth muscle and frequently present with little intervening brain parenchyma.

Microscopically, cavernous angiomas consist of sclerotic, variably calcified compact vessels in a honeycomb pattern.

Unlike telangiectasis and arteriovenous malformation these lesions do not contain interstitial parenchyma.

The cells that form the vessels do not form the necessary junctions with surrounding cells and the structural support from the smooth muscle is hindered causing leakage into the surrounding tissue.

Clinical features

It is the leakage of blood, known as a hemorrhage from these vessels that causes a variety of symptoms known to be associated with this disease.

Diagnosis

Cavernous malformation diagnosis.

Treatment

see Cavernous malformation treatment.

Outcome

When cavernous malformations can be completely removed, the risk of further growth or hemorrhage is essentially permanently eliminated 9) (however, recurrence of symptoms has been reported after partial and even seemingly-complete removal 10) 11). For CMs treated surgically, patients need to be aware that post-op neurologic worsening is very common, especially with brainstem CMs 12) Worsening may be transient, 13) but may take months to resolve.

References

1)
Cushing H, Bailey P (1928) Tumors arising from the blood-vessels of the brain. Charles C. Thomas, Springfield
2)
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.
3)
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.
4)
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.
5)
Kivelev J, Niemelä M, Hernesniemi J. Characteristics of cavernomas of the brain and spine. J Clin Neurosci. 2012;19:643–48.
6)
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.
7)
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.
8)
Gross BA, Lin N, Du R, Day AL. The natural history of intracranial cavernous malformations. Neurosurg Focus. 2011;30:E24.
9)
Wascher TM, Spetzler RF, Carter LP, Spetzler RF, Hamilton MG. In: Cavernous malformations of the brain stem. Neurovascular Surgery. New York: McGraw -Hill; 1995:541–555
10)
Zimmerman RS, Spetzler RF, Lee KS, Zabramski JM, et al. Cavernous Malformations of the Brain Stem. J Neurosurg. 1991; 75:32–39
11)
Bertalanffy H, Gilsbach JM, Eggert HR, et al. Microsurgery of deep-seated cavernous angiomas: report of 26 cases. Acta Neurochir. 1991; 108:91– 99
12)
Weil SM, Tew JM,Jr. Surgical management of brain stem vascular malformations. Acta Neurochir (Wien). 1990; 105:14–23
13)
Bartolomei J, Wecht DA, Chaloupka J, Fayad P, Awad IA. Occipital lobe vascular malformations: prevalence of visual field deficits and prognosis after therapeutic intervention. Neurosurgery. 1998; 43:415–21; discussion 421-3
cavernous_malformation.txt · Last modified: 2019/12/12 21:33 by administrador