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Paraneoplastic and autoimmune neurologic disorders are now being recognized in novel settings, including their occurrence as an immune-related adverse effect of immune checkpoint inhibitor treatment for cancer.

This article discusses when to suspect a paraneoplastic neurologic syndrome, the diagnostic utility and pitfalls of neural autoantibody testing, how to best detect the underlying tumor, and the treatment approach that involves combinations of antineoplastic treatments, immunosuppressants, and supportive/symptomatic treatments.

This article discusses when to suspect a paraneoplastic neurologic syndrome, the diagnostic utility and pitfalls of neural autoantibody testing, how to best detect the underlying tumor, and the treatment approach that involves combinations of antineoplastic treatments, immunosuppressants, and supportive/symptomatic treatments.

Management of metastasis to the central nervous system (CNS) has evolved, and molecular characterization of metastatic disease is now routinely done. Targeted therapies, once few in number with limited penetration into the CNS, have multiplied in number and increased in CNS coverage. This article addresses recent advances in the evaluation and clinical management of patients with CNS metastasis.

Metastasis of cancer to the CNS can be diagnosed and characterized with novel techniques, including molecular analyses of the spinal fluid, so-called liquid biopsies. KRIBB11 Resected parenchymal CNS metastases are now routinely subjected to genomic sequencing. For patients with CNS metastases displaying targetable mutations, a wide variety of treatment options are available, including deferral of radiation therapy in favor of a trial of an orally bioavailable targeted therapy or immunotherapy. For patients without a molecularly targetable lesion, local treatment in the form of radiation therapy, now most often stereotactic radiosurgery, is supplanting untargeted whole-brain radiation therapy.

Technologic advances in diagnosis and management have resulted in new diagnostic and therapeutic approaches to patients with metastasis to the CNS, with resulting improvements in progression-free and overall survival.

Technologic advances in diagnosis and management have resulted in new diagnostic and therapeutic approaches to patients with metastasis to the CNS, with resulting improvements in progression-free and overall survival.

This article focuses on primary brain tumors in the pediatric population with an emphasis on molecular classifications and treatment strategies.

Pediatric brain tumors are a heterogeneous group of tumors that differ from adult brain cancers despite similar nomenclature. With the added complexity of the developing brain, treatment regimens are tailored to protect neurocognitive outcomes without sacrificing long-term survival. The 2016 World Health Organization's classification incorporated molecular characteristics to aid in defining the diagnosis and prognosis of these tumors. These changes have enabled providers to stratify patients, thus intensifying therapies in those with high-risk diseases and modifying treatments to reduce morbidity for children and to provide better outcomes. Recent published findings from clinical trials have been especially helpful for gliomas, embryonal tumors, and ependymomas. By using this new information, molecular factors that correlate with survival have been identified in patients. In addition, genetic findings in tumor tissue have also led to revelations in predisposing germline mutations.

New findings from clinical trials and molecular stratification will shape the next generation of therapies in hopes of improving overall outcome, identifying pathways in tumorigenesis, and aiding in genetic counseling for children and their families.

New findings from clinical trials and molecular stratification will shape the next generation of therapies in hopes of improving overall outcome, identifying pathways in tumorigenesis, and aiding in genetic counseling for children and their families.

Although sporadic primary neoplasms account for the majority of nervous system tumors, familial nervous system tumor syndromes are important and clinically relevant conditions for the neurologist to understand. This article reviews common inherited nervous system tumor syndromes including neurofibromatosis type 1, neurofibromatosis type 2, schwannomatosis, tuberous sclerosis complex, and von Hippel-Lindau syndrome. The epidemiology, genetics, approach to diagnosis, neurologic and nonneurologic manifestations, and management options are reviewed.

Awareness of the more common and clinically relevant familial nervous system tumor syndromes is important. These conditions teach us about the underlying biology that drives tumor development in the central and peripheral nervous systems including peripheral nerve sheath tumors (eg, neurofibroma, schwannoma), meningioma, vestibular schwannoma, subependymal giant cell astrocytoma, and hemangioblastoma. Knowledge of the clinical manifestations ensures that the neurosurgeons, radiation oncologists, otolaryngologists, pathologists, neuropsychologists, physical medicine and rehabilitation specialists, and geneticists is necessary for the optimal treatment of these patients.

This article describes the diagnosis and management of meningioma, pituitary adenoma, craniopharyngioma, and glioneuronal tumors.

Both meningiomas and pituitary adenomas are common brain tumors. In many cases, these lesions are found incidentally on imaging when patients are being evaluated for a variety of symptoms and signs. While nonmalignant, these tumors are occasionally associated with significant morbidity due to location and resulting secondary symptoms. Rarely, these tumors can also transform into malignant variants. Surgical techniques allow for more complete resections with minimal complications. Significant progress is being made in understanding the molecular biology of meningioma, which may result in wider availability of targeted therapies, especially for patients who are not candidates for other therapeutic modalities. Medical therapies for secretory pituitary adenomas continue to evolve. Craniopharyngiomas are nonmalignant tumors associated with significant morbidity due to their location.

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