From the Guidelines
Cerebral cavernous hemangioma and cavernous malformation are actually the same condition, just referred to by different names. These terms both describe abnormal clusters of blood vessels in the brain that form dilated, blood-filled spaces called caverns. These lesions have thin walls without normal vessel structure and contain blood at various stages of thrombosis and organization. Other synonyms for this condition include cavernous angioma, cavernoma, and cerebral cavernous malformation (CCM) 1. The condition is characterized by its raspberry-like appearance on imaging and can cause symptoms including seizures, headaches, neurological deficits, or hemorrhage, though many remain asymptomatic.
Key Characteristics
- The condition can be sporadic or familial, with the familial form linked to mutations in three genes: CCM1 (KRIT1), CCM2, and CCM3 (PDCD10) 1.
- Cavernomas are comprised of endothelial spaces containing venous blood and account for 17% of hemorrhagic stroke in children 1.
- Patients with cavernoma (15%) have multiple lesions and 10% have a family history of cavernoma and may have mutations in CCM1, CCM2, and CCM3 genes 1.
- Although 9% are associated with prior cranial irradiation, 86% are supratentorial and 14% infratentorial in location 1.
- Cavernomas have an annual hemorrhage rate of 3.3% to 4.5% 1.
Treatment Options
Treatment options depend on symptoms, location, and bleeding risk, ranging from observation to surgical removal or stereotactic radiosurgery. Understanding that these terms refer to the same entity helps avoid confusion when reviewing medical literature or discussing treatment options with healthcare providers. According to the most recent study 1, the management of cavernous malformations should prioritize minimizing the risk of hemorrhage and improving quality of life.
Diagnosis and Imaging
Diagnosis is often made using MRI with T2-weighted gradient-echo sequences, which more reliably identifies less obvious cavernous malformations than other MRI sequences or CT 1. The characteristic “bull’s-eye” appearance of a cavernous malformation on MRI is due to bleeding and residual hemosiderin deposition 1.
From the Research
Definition and Characteristics
- Cerebral cavernous malformations (CMs) are vascular lesions found in the central nervous system (CNS) and throughout the body, also known as cavernomas, cavernous angiomas, and cavernous hemangiomas 2.
- CMs are angiographically occult, meaning they are not visible on angiography, and their diagnosis relies on various MR imaging techniques 3, 4.
- Cavernous hemangiomas, on the other hand, are benign vascular tumors that occur within the cavernous sinus (CS) and are different from CMs clinically, on imaging studies, and in their response to treatment 5.
Differences between Cerebral Cavernous Hemangioma and Cavernous Malformation
- CMs are true vascular malformations, whereas hemangiomas are benign vascular tumors 5.
- The term "cavernous malformation" refers to intraaxial lesions only, while "cavernous sinus hemangioma" refers to extraaxial, intradural hemangiomas of the CS 5.
- Cavernous angioma (CA) is also known as cavernoma, cavernous hemangioma, and cerebral cavernous malformation (CCM), and can occur as a solitary hemorrhagic vascular lesion or as clustered lesions associated with a developmental venous anomaly 6.
Clinical Presentation and Treatment
- CMs can present clinically with seizures, focal neurological deficits, and/or hemorrhage 3, 4.
- The clinical presentation and location of the lesion are the most important factors involved in determining the optimal course of treatment of CMs 3.
- Surgical resection is the gold standard treatment for surgically-accessible, symptomatic CMs, while incidental CMs or minimally symptomatic, surgically inaccessible eloquent lesions may be considered for observation 4.