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Gangliocytoma of central nervous system

MedGen UID:
1681593
Concept ID:
C5190517
Neoplastic Process
SNOMED CT: Gangliocytoma of central nervous system (782680004)

Definition

A rare mixed neuronal-glial tumour characterised by slow growth and irregular arrangement of neoplastic ganglion cells (large, multipolar dysplastic neurons) within stroma composed of non-neoplastic glial elements. Most commonly it occurs in temporal lobe, but it can be located throughout central nervous system. Clinical manifestations vary depending on the location and include seizures, increased intracranial pressure, cerebellar signs and focal neurologic deficits. Memory disturbances, cranial nerve palsies and psychiatric symptoms have also been reported. [from SNOMEDCT_US]

Professional guidelines

PubMed

Vidhya Annapoorni CS, Retnaswami CS, Mailankody P, Katragadda P, Pillai SK, Rangarajan A, Padmanabha H, Pendharkar H
Neurol India 2020 Mar-Apr;68(2):368-372. doi: 10.4103/0028-3886.280647. PMID: 32415012

Recent clinical studies

Therapy

Tan TSE, Sanamandra SK
Postgrad Med J 2021 Feb;97(1144):125-126. Epub 2020 May 13 doi: 10.1136/postgradmedj-2020-137789. PMID: 32404494
Zak M, Ledbetter M, Maertens P
J Child Neurol 2017 Mar;32(3):322-326. Epub 2016 Dec 8 doi: 10.1177/0883073816681340. PMID: 27932596
Sone D, Ito K, Taniguchi G, Murata Y, Nakata Y, Watanabe Y, Okazaki M, Sato N, Matsuda H, Watanabe M
Epilepsy Res 2015 May;112:114-21. Epub 2015 Mar 10 doi: 10.1016/j.eplepsyres.2015.02.018. PMID: 25847346
Aguilar HN, Hung RW, Mehta V, Kotylak T
J Radiol Case Rep 2012 Oct;6(10):1-10. Epub 2012 Oct 1 doi: 10.3941/jrcr.v6i10.1134. PMID: 23378871Free PMC Article
Freeman CR, Souhami L, Caron JL, Villemure JG, Olivier A, Montes J, Farmer JP, Podgorsak EB
Med Pediatr Oncol 1994;22(3):173-80. doi: 10.1002/mpo.2950220305. PMID: 8272006

Prognosis

Harvengt J, Gernay C, Mastouri M, Farhat N, Lebrethon MC, Seghaye MC, Bours V
J Clin Endocrinol Metab 2020 Jul 1;105(7) doi: 10.1210/clinem/dgaa247. PMID: 32407531
Ho CY, Bornhorst M, Almira-Suarez MI, Donev K, Grafe M, Gordish-Dressman H, Rodriguez FJ
J Neuropathol Exp Neurol 2020 Jan 1;79(1):67-73. doi: 10.1093/jnen/nlz115. PMID: 31793986Free PMC Article
Xu T, Zhu W, Wang P
Medicine (Baltimore) 2019 Apr;98(15):e15203. doi: 10.1097/MD.0000000000015203. PMID: 30985715Free PMC Article
Khandpur U, Huntoon K, Smith-Cohn M, Shaw A, Elder JB
World Neurosurg 2019 Jul;127:319-325. Epub 2019 Mar 21 doi: 10.1016/j.wneu.2019.03.131. PMID: 30905649
Sarnat HB
J Child Neurol 2000 Oct;15(10):675-87. doi: 10.1177/088307380001501007. PMID: 11063082

Clinical prediction guides

Gambini D, Ferrero S, Bulfamante G, Pisani L, Corbo M, Kuhn E
Neuropathol Appl Neurobiol 2024 Apr;50(2):e12970. doi: 10.1111/nan.12970. PMID: 38504418
Ho CY, Bornhorst M, Almira-Suarez MI, Donev K, Grafe M, Gordish-Dressman H, Rodriguez FJ
J Neuropathol Exp Neurol 2020 Jan 1;79(1):67-73. doi: 10.1093/jnen/nlz115. PMID: 31793986Free PMC Article
Xu T, Zhu W, Wang P
Medicine (Baltimore) 2019 Apr;98(15):e15203. doi: 10.1097/MD.0000000000015203. PMID: 30985715Free PMC Article
Krejs GJ
Am J Med 1987 May 29;82(5B):37-48. doi: 10.1016/0002-9343(87)90425-6. PMID: 3035922
King D, Goodman J, Hawk T, Boles ET Jr, Sayers MP
Arch Surg 1975 Aug;110(8):888-91. doi: 10.1001/archsurg.1975.01360140032006. PMID: 1171673

Recent systematic reviews

Harvengt J, Gernay C, Mastouri M, Farhat N, Lebrethon MC, Seghaye MC, Bours V
J Clin Endocrinol Metab 2020 Jul 1;105(7) doi: 10.1210/clinem/dgaa247. PMID: 32407531

Supplemental Content

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