Rare Neuronal, Glial and Glioneuronal Tumours in Adults
Abstract
Simple Summary
Abstract
1. Introduction
2. Neuronal Tumours
2.1. Dysplastic Cerebellar Gangliocytoma (Lhermitte–Duclos Disease, DCG)
2.2. Central Neurocytoma
2.3. Extraventricular Neurocytoma
3. Glioneuronal Tumours
Tumour | Median Age (Years)/ Sex | Localisation | Immunohisto Chemistry | Specific Mutations | Histology | Symptoms | Treatment | MRI Particularities |
---|---|---|---|---|---|---|---|---|
Multinodular and vacuolating neuronal tumours | 40 y, M > F [31] | Temporal/frontal lobes, | OLIG2+, synaptophysin-, CD34-, GFAP-. NeuN- [32] | MAPK pathway mutations of MAP2K1 and of BRAF (excluding BRAFV600E) [33]. FGFR2 fusion | Purely neuronal (non-neurocytic and no neoplastic glial cells). Absence of mitoses. | Mainly seizures [34] Sometimes incidental finding. | Observation preferred. Surgery only if refractory epilepsy [35]. Molecular targeted therapy *. | Small superficial cortical cystic lesions, sometimes in clusters [31]. |
Rosette-forming glioneuronal tumour (Figure 2) | 20 y | Midline structures in proximity of the 4th ventricle and the aqueduct of Sylvius | Neurocytes: Olig2+, rosettes: synaptophysin+, Glial cells: GFAP+, S100+ | FGFR1 mutations are very common, associated with PIK3CA, PIK3R1 or NF1 mutations [36] | Biphasic tumour with a component of neurocytes forming rosettes and/or pseudorosettes, and a glial component (often pilocytic astrocytes). | Progressive brainstem/cerebellar signs and visual disturbance. | Surgical resection is preferred [37]. If aggressive features and/or leptomeningeal infiltration, spinal metastasis: RT and chemotherapy [38] can be discussed in an adjuvant manner after surgery. Molecular targeted therapy*. | Mix of cystic and solid lesions, strong gadolinium enhancement. “Green bell pepper sign” [39]. |
Papillary glioneuronal tumour | 25 y | Supratentorial: mainly temporal and frontal lobes | Neurocytes: Olig2+, synaptophysin+, Astrocytic cells of papilla: GFAP+ | PRKCA gene fusions, mostly SLC44A1:PRKCA fusion [40,41] | biphasic organisation with astrocytic papillas around hyalinized vessels and a neuronal component (most often neurocytic). | Headaches [42] and seizures. Incidental finding if small enough. Often characterized by an indolent course [43]. | Surgical resection alone is preferred: with very rare recurrences [44]. RT and/or chemotherapy if high Ki-67 in recurrence or other features of aggressiveness [45]. | Solid and a cystic component [46]. Septations can be quite specific. Calcifications are frequent. |
Myxoid glioneuronal tumour (previously DNT of the septum pellucidum) | 20–25 years | Septum pellucidum, periventricular locations, corpus callosum [47]. | OLIG2+, SOX10+, GFAP+ | dinucleotide mutation at codon p.K385 in the PDGFRA gene [47] | Histologically similar to DNT or RGNT. | Hydrocephalus the most frequent initial clinical presentation, incidental findings not rare. | Gross surgical resection alone is preferred. In case of relapse and/or dissemination, RT [48] and/or chemotherapy (TMZ, CCNU) [47] can be considered. | No contrast enhancement, nor diffusion restriction. Partially suppressed Flair in centre, no oedema [48,49]. |
Gangliocytoma | Children, young adults | Mainly temporal lobe [50]. Sellar locations also seen [51]. | chromogranin A+, synaptophysin+, neurofilament+, GFAP- | BRAFV600E mutation of alternative MAPK pathway alterations [52]. | multinucleated ganglionic neuronal tumour cells. | Seizures due to temporal/cortical locations [50,53]. Sometimes headaches, brainstem signs. | Surgical resection [53]. Relapse after resection remains very rare [54]. Chemotherapy has no place, neither radiotherapy. Molecular targeted therapy*. | Strong Gd enhancement, cystic images, perilesional oedema, calcifications [54]. |
Diffuse glioneuronal tumour with oligodendroglioma-like features and nuclear clusters | Young adults, children | Supratentorial locations. | Synaptophysin+, NeuN+, MAP2+, Olig2+, GFAP- | Monosomy 14. Distinct methylation profile [55]. | Pseudo-oligodendroglial cells infiltrating cerebral cortex and forming nuclear clusters. Low number of mitosis. | Unspecific. | No standard treatment. Gross total surgery followed by radiotherapy may be a good option [56]. | None or only discrete Gd enhancement, no oedema. |
3.1. Ganglioglioma
3.2. Dysembryoplastic Neuroepithelial Tumour (DNET)
3.3. Diffuse Leptomeningeal Glioneuronal Tumour (DLGNT)
3.4. Cerebellar Liponeurocytoma
3.5. Subependymal Giant Cell Astrocytoma (SEGA)
4. Glial Tumours
4.1. Pilocytic Astrocytomas
4.2. High-Grade Astrocytoma with Piloid Features (HGAP)
4.3. Astroblastoma MN1 Altered
4.4. Chordoid Gliomas
4.5. Pleomorphic Xanthoastrocytoma (PXA)
5. Conclusions
Author Contributions
Funding
Acknowledgments
Conflicts of Interest
References
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Crainic, N.; Furtner, J.; Pallud, J.; Bielle, F.; Lombardi, G.; Rudà, R.; Idbaih, A. Rare Neuronal, Glial and Glioneuronal Tumours in Adults. Cancers 2023, 15, 1120. https://doi.org/10.3390/cancers15041120
Crainic N, Furtner J, Pallud J, Bielle F, Lombardi G, Rudà R, Idbaih A. Rare Neuronal, Glial and Glioneuronal Tumours in Adults. Cancers. 2023; 15(4):1120. https://doi.org/10.3390/cancers15041120
Chicago/Turabian StyleCrainic, Nicolas, Julia Furtner, Johan Pallud, Franck Bielle, Giuseppe Lombardi, Roberta Rudà, and Ahmed Idbaih. 2023. "Rare Neuronal, Glial and Glioneuronal Tumours in Adults" Cancers 15, no. 4: 1120. https://doi.org/10.3390/cancers15041120
APA StyleCrainic, N., Furtner, J., Pallud, J., Bielle, F., Lombardi, G., Rudà, R., & Idbaih, A. (2023). Rare Neuronal, Glial and Glioneuronal Tumours in Adults. Cancers, 15(4), 1120. https://doi.org/10.3390/cancers15041120