Skull Base Tumors

• Published in: Continuum (Minneapolis, Minn.) Vol. 29; no. 6; p. 1752
• Main Authors: Forst, Deborah A, Jones, Pamela S
• Format: Journal Article
• Language: English
• Published: United States 01.12.2023
• Subjects: Humans; Meningeal Neoplasms - diagnosis; Meningeal Neoplasms - genetics; Meningeal Neoplasms - therapy; Neuroendocrine Tumors - surgery; Neurosurgical Procedures; Pituitary Neoplasms - surgery; Skull Base - pathology; Skull Base - surgery; Skull Base Neoplasms - diagnosis; Skull Base Neoplasms - genetics; Skull Base Neoplasms - therapy
• ISSN: 1538-6899
• DOI: 10.1212/CON.0000000000001361

This article reviews the presenting features, molecular characteristics, diagnosis, and management of selected skull base tumors, including meningiomas, vestibular schwannomas, pituitary neuroendocrine tumors, craniopharyngiomas, chordomas, ecchordosis physaliphora, chondrosarcomas, esthesioneuroblastomas, and paragangliomas. Skull base tumors pose a management challenge given their complex location and, as a result, the tumors and treatment can result in significant morbidity. In most cases, surgery, radiation therapy, or both yield high rates of disease control, but the use of these therapies may be limited by the surgical accessibility of these tumors and their proximity to critical structures. The World Health Organization classification of pituitary neuroendocrine tumors was updated in 2022. Scientific advances have led to an enhanced understanding of the genetic drivers of many types of skull base tumors and have revealed several potentially targetable genetic alterations. This information is being leveraged in the design of ongoing clinical trials, with the hope of rendering these challenging tumors treatable through less invasive and morbid measures. Tumors involving the skull base are heterogeneous and may arise from bony structures, cranial nerves, the meninges, the sinonasal tract, the pituitary gland, or embryonic tissues. Treatment often requires a multidisciplinary approach, with participation from radiation oncologists, medical oncologists, neuro-oncologists, and surgical specialists, including neurosurgeons, otolaryngologists, and head and neck surgeons. Treatment has largely centered around surgical resection, when feasible, and the use of first-line or salvage radiation therapy, with chemotherapy, targeted therapy, or both considered in selected settings. Our growing understanding of the molecular drivers of these diseases may facilitate future expansion of pharmacologic options to treat skull base tumors.