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Seizures and AVMs

Pearls Presentation of iAVMs with seizures is more common in adult than in pediatric populations. AVMs in the frontal, temporal, and parietal lobes more commonly are associated with seizures. Size (> 3–4 cm), cortical location, long arterial feeders of middle cerebral artery origin, and superficial venous drainage with varicosities and tortuosity are risk factors for seizures. Surgical removal of iAVMs, with or without adjunctive embolization, is…

Classification Systems

Pearls Multimodality intervention for iAVM therapy complicates the specific surgical, endovascular, and radiosurgical risk-assessment systems. The most commonly applied classification for assessing surgical risk is the Spetzler-Martin grading system ( Table 8.1 ). Table 8.1 Surgical iAVM Classifications a Classification Grades Points Per Covariate Covariates Categorical Covariate Points Categorical Point Definition Spetzler-Martin grading system I–V 1 Eloquence 1 Deep venous drainage 3 Size 1 < 3 cm…

Hemodynamic Factors: Steal/Breakthrough Bleeding

Pearls Abnormal vessels in the iAVM nidus allow direct transmission of high-pressure flow into venous low-pressure vessels, thereby increasing bleeding risk. Decreased resistance in abnormal iAVM vessels allows blood to flow into them more easily and “steal” blood flow from the surrounding brain, causing neurologic deficits. Venous variations secondary to iAVMs—dilation, varices, or stenoses—lead to increased risk for bleeding. Bleeding after successful iAVM resection may be…

Aneurysms Associated With AVMs

Pearls AVM-associated aneurysms are reported in 10%–20% of iAVM cases. Aneurysm size and number correlate with increasing patient age. Aneurysms are more commonly described in association with posterior fossa AVMs. The rupture rate of aneurysms associated with iAVMs is higher (7% annually). Intranidal aneurysms are resected with the iAVM; proximal flow-related aneurysms often regress after treatment of the AVM. Prevalence/Demographics Intracranial arteriovenous malformations (iAVMs) are frequently…

Natural History of Intracranial AVMs

Pearls Common presentations of iAVMs include hemorrhage, seizure, headache, and focal neurological deficit. The risk of iAVM bleeding is increased with deep location, venous outflow obstruction, associated aneurysms, and deep venous drainage. Small iAVMs may have an increased risk for bleeding, but anatomical factors must also be considered. The annual bleeding risk for an iAVM is generally quoted at between 2% and 4% but may be…

MRI Neurovascular Evaluation: Blood Flow, Perfusion, Diffusion, and Susceptibility

Pearls Advanced MRI techniques, including 4D flow, arterial spin labeling, diffusion, and susceptibility imaging, expand conventional anatomic assessments to physiologic evaluation. Understanding the underlying physics and properties of these sequences informs their complementary application in the assessment of iAVMs, including detailed evaluation of blood flow, parenchymal perfusion, fiber tract injury and location, and the presence of extravascular blood products. Improvements in these MRI-based techniques have allowed…

Radiographic Anatomy: CT/MRI/Angiography and Risks

Pearls Imaging plays an integral role in detection, grading, and management of iAVMs. Intracranial hemorrhage in a young patient should raise suspicion of an underlying iAVM. CT angiography is 90% sensitive in the detection of iAVMs. 4D contrast-enhanced MR angiography at 3.0 T has 100% agreement with digital subtraction angiography (DSA) in its ability to diagnose and classify untreated iAVMs with regard to Spetzler-Martin classification. Although…

Pathology and Genetics

Pearls AVMs can be the result of one abnormal connection (fistular AVM) or multiple abnormal connections (racemose AVM). Lack of a capillary bed in iAVMs results in abnormal anatomy in all vessels involved: arteries, nidus, and veins. AVM formation is due to a combination of dysregulated vessel formation, vascular inflammation, and abnormal vessel remodeling after injury. Risk factors for bleeding from an iAVM: previous hemorrhage, deep…

Anatomy and Histology of Intracranial AVMs

Pearls Intracranial AVMs are focal abnormal conglomerations of dilated arteries and veins that pulsate in the sulci and gyri of the brain and can cause stroke if they rupture. The pathogenesis of iAVMs remains poorly understood, and traditional theories regarding the congenital etiology of these lesions are being challenged and replaced by more comprehensive pathophysiological hypotheses. Features such as exclusive deep venous drainage, deep brain location,…

Lessons Learned: Clinical Trials and Other Interventions for Glioblastoma

Glioblastoma (GBM) remains the most common primary malignant brain tumor in adults and accounts for 16% of all primary brain tumors and for 45% of malignant primary brain tumors. Despite ample research to find better treatments the prognosis of GBM remains grim, with a less than 10% 5-year survival with maximal medical treatment. In 2004, the European Organization for Research and Treatment of Cancer and National…

National and Global Economic Impact of Glioblastoma

Introduction The cost of cancer treatment has progressively increased over the past decade as newer chemotherapy and immunotherapy agents have been established. Glioblastoma, in particular, has a significant cost in the health care system because of both the severity of neurologic dysfunction that is associated with the disease and the wide range of treatment options offered in order to prolong survival. The costs of glioblastoma can…

Socioeconomics and Survival

Introduction Accounting for 52% of all primary brain tumors, glioblastoma is the most common and most aggressive. Glioblastomas account for 20% of all intracranial tumors. Disparities in risk factors, incidence, treatment, and follow-up have been shown in the literature for several types of cancer. For patients with glioblastoma, the influence of socioeconomic factors, including gender, race, ethnicity, income level, marital status, and occupation, have been explored…

Health-related Quality of Life and Neurocognitive Functioning After Glioblastoma Treatment

Introduction With a median survival of 12 to 14 months, the prognosis of patients with glioblastoma multiforme (GBM) remains poor. Treatment is therefore not only designed to prolong survival but also to maintain an optimal level of health-related quality of life (HRQOL). HRQOL is determined by self-report, and is a multidimensional concept. It includes people’s perception of their physical, cognitive, and affective state, as well as their…

Early Detection of Glioblastoma

Historical context and biological basis Early detection of solid tumors is vital for extending survival and possibly curing patients with cancer. This idea is especially relevant to patients with the most common and most lethal malignant brain tumor, glioblastoma (GBM), because earlier detection would likely increase not only survival but also quality of life. Historically, the more common malignancies, such as breast and colon cancer, paved the way…

General Principles of Immunotherapy for Glioblastoma

Background Despite trimodal therapy consisting of maximal safe resection and adjuvant partial radiotherapy with concurrent and subsequent temozolomide, glioblastoma (GBM) has a dismal prognosis, with a median survival of 14.6 months and an overall survival of only 9.8% at 5 years. Further contributing to therapeutic challenge is the intertumoral and intratumoral heterogeneity of GBM. The strongest predictor for response to temozolomide according to Stupp and colleagues was methylation…

Brain Plasticity and Reorganization Before, During, and After Glioma Resection

Introduction The traditional principle in neuro-oncology is to study the tumor first, with little consideration regarding the host; that is, the brain. Nevertheless, to define the optimal therapeutic management for each patient bearing a diffuse glioma (DG), the concept of oncofunctional balance must be taken into account. Although understanding of the natural history of the disease is crucial, this is not enough. The adaptive reaction of…

Tumor-Treating Electric Fields for Glioblastoma

Acknowledgments The authors acknowledge and thank Kisa Zhang for her artwork in Fig. 17.1 . Historical context of electric field treatment The application of physical energy from various parts of the electromagnetic spectrum is common in glioblastoma treatment. The most widely used involves energies from the higher end of the spectrum in exahertz (the 10 18 -Hz range), in which ionizing radiation is used to treat various…

Local Drug Delivery in the Treatment of Glioblastoma

The prognosis following diagnosis of glioblastoma remains poor. Historically, there have been many notable attempts to use local drug delivery to treat glioblastoma, including convection-enhanced delivery (CED), direct tumor injection, and the use of to deliver chemotherapeutics. The use of polymer wafers resulted in the only US Food and Drug Administration (FDA)–approved intracranial drug implant for treatment of recurrent and de novo glioblastoma, Gliadel. Over a…

Minimally Invasive Targeted Therapy for Glioblastoma: Laser Interstitial Thermal Therapy

Introduction Laser interstitial thermal therapy (LITT) is a minimally invasive treatment modality for brain tumors and other central nervous system (CNS) disorders, first introduced by Bown in 1983, which has been revived over the last 2 decades because of recent technological advancements in laser technology and MRI thermography. At that time, the main limitations of this surgical technique were the inability to monitor or predict the…

Intraoperative Imaging of Glioblastoma

Introduction Glioblastoma (GBM) is one of the deadliest forms of cancer, with a median survival of approximately 15 months. Standard of care treatment of GBM includes maximal safe surgical resection, fractionated radiotherapy, temozolomide chemotherapy, and, most recently, tumor treatment fields. Despite this combination treatment regimen, tumor progression or recurrence occurs after a median of 7 months. Overall survival of patients with GBM is determined by multiple factors, including…