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Uncommon brain tumors

Authors
Andrew D Norden, MD
Milan G Chheda, MD
Patrick Y Wen, MD
Section Editor
Jay S Loeffler, MD
Deputy Editor
April F Eichler, MD, MPH

INTRODUCTION

Brain tumors are a diverse group of neoplasms arising from different cells within the central nervous system (CNS) or from systemic tumors that have metastasized to the CNS (table 1) [1,2]. The most common of these include the gliomas and meningiomas, as well as metastatic lesions from extracranial tumors. (See "Classification and pathologic diagnosis of gliomas".)

The clinical manifestations of brain tumors are determined by the anatomic location of the lesion and its rate of growth, as well as the histology of the specific tumor. Although symptoms, imaging characteristics, and demographic features may suggest a specific tumor type, definitive diagnosis of a brain tumor requires biopsy and in many instances, molecular studies for confirmation. (See "Clinical presentation and diagnosis of brain tumors".)

For the rare brain tumors that are reviewed here, information about the clinical and imaging manifestations, natural history, and management is based upon case reports and small series. Recommendations regarding treatment are extrapolated from these limited data and from general principles of brain tumor management. More detailed discussions of other tumor types are presented separately (see specific topic reviews).

NEURONAL AND NEURONOGLIAL TUMORS

Rare tumors characterized by a variable degree of neuronal differentiation include gangliogliomas and gangliocytomas, dysplastic gangliocytoma of the cerebellum (Lhermitte Duclos disease), desmoplastic infantile astrocytoma/ganglioglioma, cerebellar liponeurocytomas, central and extraventricular neurocytomas, papillary glioneuronal tumors, rosette-forming glioneuronal tumor of the fourth ventricle, and glomus tumors (paragangliomas).

Ganglioglioma and gangliocytoma — Gangliogliomas and gangliocytomas comprise a spectrum of low-grade tumors characterized by a neoplastic neuronal population. In gangliogliomas, the neoplastic neuronal cells are accompanied by neoplastic glial cells, while in gangliocytomas, large, well-differentiated neurons are the sole neoplastic component. Although these tumors may arise anywhere in the neuraxis, most are supratentorial and located in the temporal lobes. Up to 60 percent of gangliogliomas harbor a BRAF V600E mutation [3,4]. The mutated protein is found primarily in the neuronal cells [3]. Mutations in isocitrate dehydrogenase 1 (IDH1), when found in a few tumors histologically identified as gangliogliomas, are associated with an older age at diagnosis, a greater risk of recurrence, and a poor prognosis [5].

                                         

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