Abstract
Neuronal and mixed neuronal-glial tumors are a group of rare tumors of the central nervous system (CNS) characterized by either neoplastic neuronal cells or a combination of neuronal and glial neoplastic cells. Most of these tumors are benign with a favorable prognosis, although there are a minority of anaplastic and aggressive tumors. Clinical neuroradiology plays an essential role in distinguishing these benign tumors from others with a less favorable outcome. The most sensitive radiological technique for imaging these tumors is Magnetic Resonance Imaging (MRI) but Computed Tomography (CT) may be used in an emergency setting, when MRI is contraindicated, and to detect calcifications. This chapter aims to offer an overview of neuronal and mixed neuronal-glial tumors with a focus on their imaging and pathological features as well as their clinical characteristics.
This publication is endorsed by: European Society of Neuroradiology (www.esnr.org).
Similar content being viewed by others
Abbreviations
- ADC:
-
Apparent diffusion coefficient
- AGG:
-
Anaplastic ganglioglioma
- CECT:
-
Contrast-enhanced computed tomography
- Cho:
-
Choline
- CN:
-
Central neurocytoma
- CNS:
-
Central nervous system
- CSF:
-
Cerebrospinal fluid
- CT:
-
Computed tomography
- DCG:
-
Dysplastic gangliocytoma of the cerebellum (Dysplastic cerebellar gangliocytoma)
- DIA:
-
Desmoplastic infantile astrocytoma
- DIG (or DIGG):
-
Desmoplastic infantile ganglioglioma
- DLGNT:
-
Diffuse leptomeningeal glioneuronal tumor
- DNT (or DNET):
-
Dysembryoplastic neuroepithelial tumor
- DWI:
-
Diffusion-weighted imaging
- EVN:
-
Extraventricular neurocytoma
- FCD:
-
Focal cortical dysplasia
- FLAIR:
-
Fluid attenuated inversion recovery
- GC:
-
Gangliocytoma
- GFAP:
-
Glial fibrillary acidic protein
- GG:
-
Ganglioglioma
- GM:
-
Gray matter
- GRE:
-
Gradient-recalled echo
- IDH:
-
Isocitrate dehydrogenase
- iMRI:
-
Intraoperative MRI
- LDD:
-
Lehrmitte-Duclos disease
- MAP 2 K1:
-
Mitogen-activated protein kinase 1
- MB:
-
Medulloblastoma
- MIBG:
-
Metaiodobenzylguanidine
- MRI:
-
Magnetic resonance imaging
- MVNT:
-
Multinodular and vacuolating neuronal tumor of the cerebrum
- NAA:
-
N-AcetylAspartate
- NECT:
-
Non-enhanced computed tomography
- NeuN:
-
Neuronal nuclear protein
- NF-1:
-
Neurofibromatosis type 1
- PGL:
-
Paraganglioma
- PGNT:
-
Papillary glioneuronal tumor
- PNET:
-
Primitive neuroectodermal tumor
- PTEN:
-
Phosphatase and tensin homologue
- PXA:
-
Pleomorphic xanthoastrocytoma
- RGNT:
-
Rosette-forming glioneuronal tumor
- SDH:
-
Succinate dehydrogenase
- SHH:
-
Sonic hedgehog
- SWI:
-
Susceptibility-weighted imaging
- TLE:
-
Temporal lobe epilepsy
- WHO:
-
World Health Organisation
- WM:
-
White matter
References
Causil LD, Ames R, Puac P, Castillo M. Adult brain tumors and Pseudotumors: Interesting (Bizarre) Cases. Neuroimaging Clin N Am. 2016;26:667–89.
Gembruch O, Junker A, Mönninghoff C, Ahmadipour Y, Darkwah Oppong M, Sure U, El Hindy N, Lemonas E. Liponeurocytoma: systematic review of a rare entity. World Neurosurg. 2018;120:214–33.
Hsu C, Kwan G, Lau Q, Bhuta S. Rosette-forming glioneuronal tumour: imaging features, histopathological correlation and a comprehensive review of literature. Br J Neurosurg. 2012;26:668–73.
Louis DN, Ohgaki H, Wiestler OD, Cavenee WK (2016). WHO classification of tumours of the central nervous system. IARC, Lyon: 2016.
Lyle MR, Dolia JN, Fratkin J, Nichols TA, Herrington BL. Newly identified characteristics and suggestions for diagnosis and treatment of diffuse leptomeningeal Glioneuronal/Neuroepithelial tumors: a case report and review of the literature. Child Neurol Open. 2015;2. https://doi.org/10.1177/2329048X14567531
Newton HB, Dalton J, Ray-Chaudhury A, Gahbauer R, McGregor J. Aggressive papillary glioneuronal tumor: case report and literature review. Clin Neuropathol. 2008;27:317–24.
Sweiss FB, Lee M, Sherman JH. Extraventricular neurocytomas. Neurosurg Clin N Am. 2015;26:99–104.
Thom M, Liu J, Bongaarts A, Reinten RJ, Paradiso B, Jäger HR, Reeves C, Somani A, An S, Marsdon D, McEvoy A, Miserocchi A, Thorne L, Newman F, Bucur S, Honavar M, Jacques T, Aronica E. Multinodular and vacuolating neuronal tumors in epilepsy: dysplasia or neoplasia. Brain Pathol. 2018;28:155–71.
Further Reading
Bourekas EC, Bell SD, Ladwig NR, Gandhe AR, Shilo K, McGregor JM, Lehman NL, Newton HB. Anaplastic papillary glioneuronal tumor with extraneural metastases. J Neuropathol Exp Neurol. 2014;73:474–6.
Cathcart SJ, Klug JR, Helvey JT, L White M, Gard AP, McComb RD. Multinodular and Vacuolating neuronal tumor: a rare seizure-associated entity. Am J Surg Pathol. 2017;41:1005–10.
Choi E, Kim SI, Won JK, Chung CK, Kim SK, Choi SH, Choi S, Han B, Ahn B, Sun-Wha IM, Park SH. Clinicopathological and molecular analysis of multinodular and vacuolating neuronal tumors of the cerebrum. Hum Pathol. 2018;86:203.
Fujita Y, Kinoshita M, Ozaki T, Kitamura M, Nakatsuka SI, Kanemura Y, Kishima H. Enlargement of papillary glioneuronal tumor in an adult after a follow-up period of 10 years: a case report. J Surg Case Rep. 2018;2018:rjy123.
Heijink DS, Urgun K, Sav A, Seker A, Konya D. A case of primary diffuse leptomeningeal gliomatosis predominantly involving the cervical spinal cord and mimicking chronic meningitis. Turk Neurosurg. 2012;22:90–4.
Husain N, Husain M. Endoscopic diagnosis of a pineal papillary glioneuronal tumor with extensive ventricular involvement: case report with review of literature. Neurol India. 2009;57:792–5.
Javahery RJ, Davidson L, Fangusaro J, Finlay JL, Gonzalez-Gomez I, McComb JG. Aggressive variant of a papillary glioneuronal tumor. Report of 2 cases. J Neurosurg Pediatr. 2009;3:46–52.
Jenkinson MD, Bosma JJ, Du Plessis D, Ohgaki H, Kleihues P, Warnke P, Rainov NG. Cerebellar liponeurocytoma with an unusually aggressive clinical course: case report. Neurosurgery. 2003;53:1425–7.
Kaloostian PE, Chen H, Tran HP. Malignant papillary glioneuronal tumor of the pineal gland: case presentation and literature review of a distinct entity. Am J Case Rep. 2013;14:164–8.
Kang JH, Buckley AF, Nagpal S, Fischbein N, Peters KB. A diffuse leptomeningeal Glioneuronal tumor without diffuse leptomeningeal involvement: detailed molecular and clinical characterization. J Neuropathol Exp Neurol. 2018;77:751–6.
Kim JE, Lim M. Neurocytoma of the spinal cord. Neurosurg Clin N Am. 2015;26:109–15.
Komori T, Scheithauer BW, Anthony DC, Rosenblum MK, McLendon RE, Scott RM, Okazaki H, Kobayashi M. Papillary glioneuronal tumor: a new variant of mixed neuronal-glial neoplasm. Am J Surg Pathol. 1998;22:1171–83.
Kosker M, Sener D, Kilic O, Hasiloglu ZI, Islak C, Kafadar A, Batur S, Oz B, Cokugras H, Akcakaya N, Camcioglu Y. Primary diffuse leptomeningeal gliomatosis mimicking tuberculous meningitis. J Child Neurol. 2014;29:NP171–5.
Kumar M, Samant R, Ramakrishnaiah R, Fitzgerald RT, Burgin K, Van Hemert R, Angtuaco E. Rosette-forming glioneuronal tumor of the fourth ventricle. Radiol Case Rep. 2013;8:740.
Matyja E, Grajkowska W, Pucko E, Kunert P, Marchel A. Papillary glioneuronal tumor with an unusual bilateral intraventricular localization. Clin Neuropathol. 2015;34:6–12.
Maxwell JE, Howe JR. Imaging in neuroendocrine tumors: an update for the clinician. Int J Endocr Oncol. 2015;2:159–68.
Nunes RH, Hsu CC, da Rocha AJ, do Amaral LLF, Godoy LFS, Watkins TW, Marussi VH, Warmuth-Metz M, Alves HC, Goncalves FG, Kleinschmidt-DeMasters BK, Osborn AG. Multinodular and Vacuolating Neuronal Tumor of the Cerebrum: A New “Leave Me Alone” Lesion with a Characteristic Imaging Pattern. AJNR Am J Neuroradiol. 2017;38:1899–1904.
Pekmezci M, Stevers M, Phillips JJ, Van Ziffle J, Bastian BC, Tsankova NM, Kleinschmidt-DeMasters BK, Rosenblum MK, Tihan T, Perry A, Solomon DA. Multinodular and vacuolating neuronal tumor of the cerebrum is a clonal neoplasm defined by genetic alterations that activate the MAP kinase signaling pathway. Acta Neuropathol. 2018;135:485–8.
Rodriguez FJ, Mota RA, Scheithauer BW, Giannini C, Blair H, New KC, Wu KJ, Dickson DW, Jenkins RB. Interphase cytogenetics for 1p19q and t(1;19)(q10;p10) may distinguish prognostically relevant subgroups in extraventricular neurocytoma. Brain Pathol. 2009;19:623–9.
Rodriguez FJ, Perry A, Rosenblum MK, Krawitz S, Cohen KJ, Lin D, Mosier S, Lin MT, Eberhart CG, Burger PC. Disseminated oligodendroglial-like leptomeningeal tumor of childhood: a distinctive clinicopathologic entity. Acta Neuropathol. 2012;124:627–41.
Ruppert B, Welsh CT, Hannah J, Giglio P, Rumboldt Z, Johnson I, Fortney J, Jenrette JM, Patel S, Scheithauer BW. Glioneuronal tumor with neuropil-like islands of the spinal cord with diffuse leptomeningeal neuraxis dissemination. J Neuro-Oncol. 2011;104:529–33.
Schlamann A, von Bueren AO, Hagel C, Zwiener I, Seidel C, Kortmann RD, Müller K. An individual patient data meta-analysis on characteristics and outcome of patients with papillary glioneuronal tumor, rosette glioneuronal tumor with neuropil-like islands and rosette forming glioneuronal tumor of the fourth ventricle. PLoS One. 2014;9:e101211.
Tsai CY, Tsai TH, Lin CH, Cheng YH, Lieu AS. Unusual exophytic neurocytoma of thoracic spine mimicking meningioma: a case report and review of the literature. Eur Spine J. 2011;20(Suppl 2):S239–42.
Author information
Authors and Affiliations
Corresponding author
Editor information
Editors and Affiliations
Section Editor information
Rights and permissions
Copyright information
© 2019 Springer Nature Switzerland AG
About this entry
Cite this entry
Carletti, F. (2019). Neuronal and Mixed Glioneuronal Tumors. In: Barkhof, F., Jager, R., Thurnher, M., Rovira Cañellas, A. (eds) Clinical Neuroradiology. Springer, Cham. https://doi.org/10.1007/978-3-319-61423-6_86-1
Download citation
DOI: https://doi.org/10.1007/978-3-319-61423-6_86-1
Received:
Accepted:
Published:
Publisher Name: Springer, Cham
Print ISBN: 978-3-319-61423-6
Online ISBN: 978-3-319-61423-6
eBook Packages: Springer Reference MedicineReference Module Medicine