Other Less Prevalent Tumors of the Central Nervous System

Adv Exp Med Biol. 2023:1405:607-643. doi: 10.1007/978-3-031-23705-8_24.


The presented tumors in this chapter are somewhat very rare, and their management is still debated due to the scarcity of information about their cell of origin, behavior, and biology. Treatment options are still limited, but we are confident that in the near future by discovering the genetic and biological mechanisms that drive tumor growth we will be able to offer new target therapies that should be flanked by surgery, radiotherapy, and chemotherapeutic agents actually in use. The purpose of this chapter is to highlight the most important known characteristics of these tumors offering the chance to recognize the disease and then offer the best opportunity for treatment to patients. The 5th WHO Classification Central Nervous System features substantial changes by moving further to advance the role of molecular diagnostics in CNS tumor classification, but remaining rooted in other established approaches to tumor characterization, including histology and immunohistochemistry, and probably, the category of many tumors will change. Here, the most important characteristics of each neoplasm are summarized focusing on genetic mechanisms and molecular pathways, their histopathologic footprints, signs and symptoms, radiologic features, therapeutic approaches, and prognosis as well as follow-up protocols. Schematic classifications are also presented to offer a better understanding of the pathology.

Keywords: Rare CNS tumors; Rare diseases; Rare entities; Rare neoplasm.

MeSH terms

  • Central Nervous System
  • Central Nervous System Neoplasms* / diagnosis
  • Central Nervous System Neoplasms* / genetics
  • Central Nervous System Neoplasms* / therapy
  • Humans