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Gangliocytoma

MedGen UID:: 1681593
•Concept ID:: C5190517
•: Neoplastic Process

Synonyms:	gangliocytoma; Gangliocytoma of central nervous system; GNC
SNOMED CT:	Gangliocytoma of central nervous system (782680004)

HPO:	HP:0034952
Monarch Initiative:	MONDO:0016730
Orphanet:	ORPHA251937

Definition

A low-grade central nervous system tumor composed of dysplastic ganglion cells, usually presenting in children or young adults and located in the cerebral hemispheres. [from HPO]

Term Hierarchy

GTR
MeSH

CClinical test, RResearch test, OOMIM, GGeneReviews, VClinVar

CROGVGangliocytoma

Disease
- Disorder by Site
  - Disorder of nervous system
    - Disorder of the central nervous system
      - Neoplasm of the central nervous system
        Neuronal/glioneuronal neoplasm of the central nervous system
        Gangliocytoma

Professional guidelines

PubMed

Clinical Characteristics, Diagnosis, and Treatment of Thyroid Stimulating Hormone-Secreting Pituitary Neuroendocrine Tumor (TSH PitNET): A Single-Center Experience.

Heo J, Suh YL, Kim SH, Kong DS, Nam DH, Lee WJ, Kim ST, Hong SD, Ryu S, Lee YB, Kim G, Jin SM, Kim JH, Hur KY
Endocrinol Metab (Seoul) 2024 Apr;39(2):387-396. Epub 2024 Feb 5 doi: 10.3803/EnM.2023.1877. PMID: 38311828 Free PMC Article

Neurosurgical treatment of gangliogliomas in children and adolescents: long-term follow-up of a single-institution series of 32 patients.

Lundar T, Due-Tønnessen BJ, Fric R, Egge A, Krossnes B, Due-Tønnessen P, Stensvold E, Brandal P
Acta Neurochir (Wien) 2018 Jun;160(6):1207-1214. Epub 2018 Apr 22 doi: 10.1007/s00701-018-3550-8. PMID: 29680921 Free PMC Article

Cancer risk and genotype-phenotype correlations in PTEN hamartoma tumor syndrome.

Nieuwenhuis MH, Kets CM, Murphy-Ryan M, Yntema HG, Evans DG, Colas C, Møller P, Hes FJ, Hodgson SV, Olderode-Berends MJ, Aretz S, Heinimann K, Gómez García EB, Douglas F, Spigelman A, Timshel S, Lindor NM, Vasen HF
Fam Cancer 2014 Mar;13(1):57-63. doi: 10.1007/s10689-013-9674-3. PMID: 23934601

See all (4)

Recent clinical studies

Etiology

Advances in Adrenal and Extra-adrenal Paraganglioma: Practical Synopsis for Pathologists.

Juhlin CC, Mete O
Adv Anat Pathol 2023 Jan 1;30(1):47-57. Epub 2022 Sep 23 doi: 10.1097/PAP.0000000000000365. PMID: 36136370

Overview of the 2022 WHO Classification of Pituitary Tumors.

Asa SL, Mete O, Perry A, Osamura RY
Endocr Pathol 2022 Mar;33(1):6-26. Epub 2022 Mar 15 doi: 10.1007/s12022-022-09703-7. PMID: 35291028

Overview of the 2022 WHO Classification of Paragangliomas and Pheochromocytomas.

Mete O, Asa SL, Gill AJ, Kimura N, de Krijger RR, Tischler A
Endocr Pathol 2022 Mar;33(1):90-114. Epub 2022 Mar 13 doi: 10.1007/s12022-022-09704-6. PMID: 35285002

Imaging of PTEN-related abnormalities in the central nervous system.

Dhamija R, Hoxworth JM
Clin Imaging 2020 Apr;60(2):180-185. Epub 2019 Dec 24 doi: 10.1016/j.clinimag.2019.12.006. PMID: 31927175

Imaging surgical epilepsy in children.

Raybaud C, Shroff M, Rutka JT, Chuang SH
Childs Nerv Syst 2006 Aug;22(8):786-809. Epub 2006 Jul 13 doi: 10.1007/s00381-006-0132-5. PMID: 16838193

See all (62)

Diagnosis

Uncommon Glioneuronal Tumors: A Radiologic and Pathologic Synopsis.

Vaz A, Cavalcanti MS, da Silva Junior EB, Ramina R, de Almeida Teixeira BC
AJNR Am J Neuroradiol 2022 Aug;43(8):1080-1089. Epub 2022 May 5 doi: 10.3174/ajnr.A7465. PMID: 35512827 Free PMC Article

Overview of the 2022 WHO Classification of Neuroendocrine Neoplasms.

Rindi G, Mete O, Uccella S, Basturk O, La Rosa S, Brosens LAA, Ezzat S, de Herder WW, Klimstra DS, Papotti M, Asa SL
Endocr Pathol 2022 Mar;33(1):115-154. Epub 2022 Mar 16 doi: 10.1007/s12022-022-09708-2. PMID: 35294740

Overview of the 2022 WHO Classification of Pituitary Tumors.

Asa SL, Mete O, Perry A, Osamura RY
Endocr Pathol 2022 Mar;33(1):6-26. Epub 2022 Mar 15 doi: 10.1007/s12022-022-09703-7. PMID: 35291028

Overview of the 2022 WHO Classification of Paragangliomas and Pheochromocytomas.

Mete O, Asa SL, Gill AJ, Kimura N, de Krijger RR, Tischler A
Endocr Pathol 2022 Mar;33(1):90-114. Epub 2022 Mar 13 doi: 10.1007/s12022-022-09704-6. PMID: 35285002

Lhermitte-Duclos disease.

Kumar R, Vaid VK, Kalra SK
Childs Nerv Syst 2007 Jul;23(7):729-32. Epub 2007 Jan 13 doi: 10.1007/s00381-006-0271-8. PMID: 17221273

See all (151)

Therapy

Securing Hemostasis in Pediatric Low-Grade Posterior Cerebral Fossa Tumors: The Value of Thrombin-Gelatin Hemostatic Matrix.

Baro V, Denaro L, d'Avella D
Pediatr Neurosurg 2018;53(5):330-336. Epub 2018 Aug 21 doi: 10.1159/000491824. PMID: 30130801

Neurosurgical treatment of gangliogliomas in children and adolescents: long-term follow-up of a single-institution series of 32 patients.

Infantile Lhermitte-Duclos Disease Treated Successfully With Rapamycin.

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

Mixed Pituitary Gangliocytoma and Prolactinoma Resistant to the Cabergoline Treatment.

Yano S, Hide T, Uekawa K, Honda Y, Mikami Y, Kuratsu JI
World Neurosurg 2016 Nov;95:620.e17-620.e22. Epub 2016 Aug 13 doi: 10.1016/j.wneu.2016.08.011. PMID: 27535625

Seizure prognosis of patients with low-grade tumors.

Kahlenberg CA, Fadul CE, Roberts DW, Thadani VM, Bujarski KA, Scott RC, Jobst BC
Seizure 2012 Sep;21(7):540-5. Epub 2012 Jun 20 doi: 10.1016/j.seizure.2012.05.014. PMID: 22726817

See all (15)

Prognosis

Advances in Adrenal and Extra-adrenal Paraganglioma: Practical Synopsis for Pathologists.

Juhlin CC, Mete O
Adv Anat Pathol 2023 Jan 1;30(1):47-57. Epub 2022 Sep 23 doi: 10.1097/PAP.0000000000000365. PMID: 36136370

Overview of the 2022 WHO Classification of Paragangliomas and Pheochromocytomas.

Mete O, Asa SL, Gill AJ, Kimura N, de Krijger RR, Tischler A
Endocr Pathol 2022 Mar;33(1):90-114. Epub 2022 Mar 13 doi: 10.1007/s12022-022-09704-6. PMID: 35285002

Clinical-pathological study of 28 glial and mixed neuronal-glial tumors diagnosed within the first year of life.

Buccoliero AM, Caporalini C, Moscardi S, Spacca B, Scagnet M, Castiglione F, Sardi I, Giunti L, Genitori L
Clin Neuropathol 2022 Jan-Feb;41(1):25-34. doi: 10.5414/NP301405. PMID: 34622774

Lhermitte-Duclos disease.

Kumar R, Vaid VK, Kalra SK
Childs Nerv Syst 2007 Jul;23(7):729-32. Epub 2007 Jan 13 doi: 10.1007/s00381-006-0271-8. PMID: 17221273

From the archives of the AFIP: superficial gliomas: radiologic-pathologic correlation. Armed Forces Institute of Pathology.

Koeller KK, Henry JM
Radiographics 2001 Nov-Dec;21(6):1533-56. doi: 10.1148/radiographics.21.6.g01nv051533. PMID: 11706224

See all (47)

Clinical prediction guides

Cerebellar phenotypes in germline PTEN mutation carriers.

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

Observation of Clinicopathologic Features of Pituitary Adenoma With Neuronal Differentiation.

Zheng L, Yan X, Hu C, Zhang P, Chen Y, Zheng Q, Hu L, Wang M, Li G, Wu P, Jiang C, Tian J, Zhang S, Wang X
Front Endocrinol (Lausanne) 2022;13:848762. Epub 2022 Mar 15 doi: 10.3389/fendo.2022.848762. PMID: 35370935 Free PMC Article

Overview of the 2022 WHO Classification of Paragangliomas and Pheochromocytomas.

Mete O, Asa SL, Gill AJ, Kimura N, de Krijger RR, Tischler A
Endocr Pathol 2022 Mar;33(1):90-114. Epub 2022 Mar 13 doi: 10.1007/s12022-022-09704-6. PMID: 35285002

Infrasellar pituitary gangliocytoma causing Cushing's syndrome.

Domingue ME, Marbaix E, Do Rego JL, Col V, Raftopoulos C, Duprez T, Vaudry H, Maiter D
Pituitary 2015 Oct;18(5):738-44. doi: 10.1007/s11102-014-0595-4. PMID: 25183169

Gangliocytomas in the sellar region.

Qiao N, Ye Z, Wang Y, Li S, Mao Y, Bao W, Che X, Qin Z, Xu W, Shen M, Chen H, Shou X, Zhao Y
Clin Neurol Neurosurg 2014 Nov;126:156-61. Epub 2014 Sep 7 doi: 10.1016/j.clineuro.2014.08.034. PMID: 25259876

See all (38)

Recent systematic reviews

Gangliocytomas of the sellar region: A challenging diagnosis.

Cossu G, Daniel RT, Messerer M
Clin Neurol Neurosurg 2016 Oct;149:122-35. Epub 2016 Aug 9 doi: 10.1016/j.clineuro.2016.08.002. PMID: 27521460

See all (1)

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Gangliocytoma

Definition

Term Hierarchy

Professional guidelines

PubMed

Recent clinical studies

Etiology

Diagnosis

Therapy

Prognosis

Clinical prediction guides

Recent systematic reviews

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Clinical resources

Practice guidelines

Consumer resources

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