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Other Less Prevalent Tumors of the Central Nervous System

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Date 2023 Jul 15
PMID 37452956
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Abstract

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.

References
1.
Abdulkader M, Mansour N, Van Gompel J, Bosh G, Dropcho E, Bonnin J . Disseminated choroid plexus papillomas in adults: A case series and review of the literature. J Clin Neurosci. 2016; 32:148-54. DOI: 10.1016/j.jocn.2016.04.002. View

2.
Abel T, Baker S, Fraser M, Tihan T, Nelson J, Yachnis A . Lhermitte-Duclos disease: a report of 31 cases with immunohistochemical analysis of the PTEN/AKT/mTOR pathway. J Neuropathol Exp Neurol. 2005; 64(4):341-9. DOI: 10.1093/jnen/64.4.341. View

3.
Adachi J, Nishikawa R, Hirose T, Matsutani M . Mixed neuronal-glial tumor of the fourth ventricle and successful treatment of postoperative mutism with bromocriptine: case report. Surg Neurol. 2005; 63(4):375-9. DOI: 10.1016/j.surneu.2004.05.039. View

4.
Ayala-Ramirez M, Feng L, Johnson M, Ejaz S, Habra M, Rich T . Clinical risk factors for malignancy and overall survival in patients with pheochromocytomas and sympathetic paragangliomas: primary tumor size and primary tumor location as prognostic indicators. J Clin Endocrinol Metab. 2010; 96(3):717-25. DOI: 10.1210/jc.2010-1946. View

5.
Badalian-Very G, Vergilio J, Degar B, Macconaill L, Brandner B, Calicchio M . Recurrent BRAF mutations in Langerhans cell histiocytosis. Blood. 2010; 116(11):1919-23. PMC: 3173987. DOI: 10.1182/blood-2010-04-279083. View