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Introduction Neurofibromatosis type 1 (NF1), neurofibromatosis type 2 (NF2), and schwannomatosis (SWN) are rare tumor suppressor conditions with estimated incidences of 1:3,000, 1:60,000, and 1:100,000, respectively. Although all three conditions are autosomal dominant and predispose to tumors throughout the central…
Introduction Leptomeningeal spread occurs in both solid tumors and hematologic malignancies. Leptomeningeal disease (LMD) for all primary cancer types portends a very poor prognosis, with survival measured in weeks if left untreated and an average of only 3.5 months with…
Introduction Brain metastases are a frequent occurrence in patients with solid malignancies. Up to 40% of patients with solid tumors will develop brain metastases at some point in their disease course, and 170,000 patients are diagnosed with brain metastases each…
Introduction Primary central nervous system lymphoma (PCNSL) is defined as lymphoma confined to the central nervous system (CNS) at presentation. By contrast, secondary CNS lymphoma represents a systemic lymphoma (i.e., outside the CNS) that has metastasized to the CNS (e.g.,…
Introduction The broad definition of high-grade glioma encompasses all central nervous system neoplasms that are derived from glial components of the brain and show anaplasia (i.e., poor cellular differentiation, atypia, pleomorphism, and loss of normal cellular organization). This chapter focuses…
Introduction The understanding of low-grade gliomas has significantly changed over the past 10 years. Genomic and epigenetic discoveries have revolutionized our understanding of these cancers. For the first time, the 2016 WHO classification of central nervous system tumors integrated molecular…
Introduction Meningiomas are slow-growing extraaxial tumors that account for about 25% of all intracranial tumors. The World Health Organization (WHO) classifies meningiomas as grade I, II, or III based upon morphologic criteria with higher grades correlating with more aggressive behavior…
Disclosures Dr. Ly has no disclosures. Dr. Jordan has research funding from the Department of Defense, the National Institutes of Health, and the Burke Foundation. He receives royalties from Elsevier and has received honoraria from the American Academy of Neurology.…
Introduction Spinal cord tumors are uncommon causes of back pain, radicular pain, and sensorimotor deficits in adults and pediatric patients. Primary spinal tumors are less common than intracranial tumors and represent approximately 2–4% of primary central nervous system (CNS) tumors.…
Introduction The posterior fossa is the infratentorial compartment of the cranial vault, which houses the cerebellum and the bulk of the brainstem. Tumors that occur in this region can arise from, or spread to, any of the adjacent structures. The…