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Chordoid glioma of the third ventricle

MedGen UID:
232956
•Concept ID:
C1322252
•
Neoplastic Process
Synonyms: Chordoid glioma; Chordoid Glioma; chordoid glioma; chordoid glioma (morphologic abnormality); Chordoid Glioma of 3rd Ventricle; chordoid glioma of 3rd ventricle; Chordoid Glioma of the 3rd Ventricle; chordoid glioma of the 3rd ventricle; Chordoid Glioma of the Third Ventricle; chordoid glioma of the third ventricle; Chordoid Glioma of the Third Ventricle (WHO Grade 2); Chordoid Glioma of the Third Ventricle (WHO Grade II); chordoid glioma of the third ventricle (WHO grade II); Chordoid Glioma of Third Ventricle; Chordoid glioma of third ventricle; chordoid glioma of third ventricle; Third Ventricle Chordoid Glioma; third ventricle chordoid glioma
SNOMED CT: Chordoid glioma of third ventricle (128789002); Chordoid glioma (128789002); Chordoid glioma (715900001)
 
Monarch Initiative: MONDO:0016706
Orphanet: ORPHA251674

Definition

An extremely rare glial neoplasm occurring in the region of the anterior third ventricle or hypothalamus, which is non-infiltrative and well-circumscribed and presents most frequently in middle-aged women with symptoms of memory loss and headaches and, because of its location, has a poor prognosis due to surgical morbidity. [from SNOMEDCT_US]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVChordoid glioma of the third ventricle

Professional guidelines

PubMed

The clinicopathological features of pituicytoma and the differential diagnosis of sellar glioma.
Wang J, Liu Z, Du J, Cui Y, Fang J, Xu L, Li G
Neuropathology 2016 Oct;36(5):432-440. Epub 2016 Feb 26 doi: 10.1111/neup.12291. PMID: 26919073
Masses and malformations of the third ventricle: normal anatomic relationships and differential diagnoses.
Glastonbury CM, Osborn AG, Salzman KL
Radiographics 2011 Nov-Dec;31(7):1889-905. doi: 10.1148/rg.317115083. PMID: 22084178

Recent clinical studies

Etiology

Suprasellar chordoid neoplasm with expression of thyroid transcription factor 1: evidence that chordoid glioma of the third ventricle and pituicytoma may form part of a spectrum of lineage-related tumors of the basal forebrain.
Hewer E, Beck J, Kellner-Weldon F, Vajtai I
Hum Pathol 2015 Jul;46(7):1045-9. Epub 2015 Mar 25 doi: 10.1016/j.humpath.2015.03.005. PMID: 25934249
Chordoid gliomas of the third ventricle share TTF-1 expression with organum vasculosum of the lamina terminalis.
Bielle F, Villa C, Giry M, Bergemer-Fouquet AM, Polivka M, Vasiljevic A, Aubriot-Lorton MH, Bernier M, Lechapt-Zalcman E, Viennet G, Sazdovitch V, Duyckaerts C, Sanson M, Figarella-Branger D, Mokhtari K; RENOP
Am J Surg Pathol 2015 Jul;39(7):948-56. doi: 10.1097/PAS.0000000000000421. PMID: 25786084
Chordoid glioma of the third ventricle.
Vanhauwaert DJ, Clement F, Van Dorpe J, Deruytter MJ
Acta Neurochir (Wien) 2008 Nov;150(11):1183-91. Epub 2008 Oct 21 doi: 10.1007/s00701-008-0014-6. PMID: 18936876
Emerging tumor entities and variants of CNS neoplasms.
Cenacchi G, Giangaspero F
J Neuropathol Exp Neurol 2004 Mar;63(3):185-92. doi: 10.1093/jnen/63.3.185. PMID: 15055442
Third ventricular chordoid glioma: case report and review of the literature.
Nakajima M, Nakasu S, Hatsuda N, Takeichi Y, Watanabe K, Matsuda M
Surg Neurol 2003 May;59(5):424-8. doi: 10.1016/s0090-3019(03)00066-1. PMID: 12765825

Diagnosis

Chordoid Glioma: A Neoplasm Found in the Anterior Part of the Third Ventricle.
Yang D, Xu Z, Qian Z, Wang L, Nie Q, Ge J, Qiu Y, Wang G
J Craniofac Surg 2021 May 1;32(3):e311-e313. doi: 10.1097/SCS.0000000000002514. PMID: 28708638
Rare glial tumors.
Soffietti R, Rudà R, Reardon D
Handb Clin Neurol 2016;134:399-415. doi: 10.1016/B978-0-12-802997-8.00024-4. PMID: 26948368
Chordoid glioma of the third ventricle: a patient presenting with SIADH and a review of this rare tumor.
Calanchini M, Cudlip S, Hofer M, Byrne J, Fabbri A, Grossman A
Pituitary 2016 Aug;19(4):356-61. doi: 10.1007/s11102-016-0711-8. PMID: 26879322
Chordoid glioma of the third ventricle: a report of two new cases, with further evidence supporting an ependymal differentiation, and review of the literature.
Pasquier B, Péoc'h M, Morrison AL, Gay E, Pasquier D, Grand S, Sindou M, Kopp N
Am J Surg Pathol 2002 Oct;26(10):1330-42. doi: 10.1097/00000478-200210000-00010. PMID: 12360048
The WHO classification of tumors of the nervous system.
Kleihues P, Louis DN, Scheithauer BW, Rorke LB, Reifenberger G, Burger PC, Cavenee WK
J Neuropathol Exp Neurol 2002 Mar;61(3):215-25; discussion 226-9. doi: 10.1093/jnen/61.3.215. PMID: 11895036

Therapy

Chordoid glioma of the third ventricle.
Vanhauwaert DJ, Clement F, Van Dorpe J, Deruytter MJ
Acta Neurochir (Wien) 2008 Nov;150(11):1183-91. Epub 2008 Oct 21 doi: 10.1007/s00701-008-0014-6. PMID: 18936876

Prognosis

Rare glial tumors.
Soffietti R, Rudà R, Reardon D
Handb Clin Neurol 2016;134:399-415. doi: 10.1016/B978-0-12-802997-8.00024-4. PMID: 26948368
Chordoid glioma of the third ventricle: a patient presenting with SIADH and a review of this rare tumor.
Calanchini M, Cudlip S, Hofer M, Byrne J, Fabbri A, Grossman A
Pituitary 2016 Aug;19(4):356-61. doi: 10.1007/s11102-016-0711-8. PMID: 26879322
Chordoid glioma of the third ventricle.
Vanhauwaert DJ, Clement F, Van Dorpe J, Deruytter MJ
Acta Neurochir (Wien) 2008 Nov;150(11):1183-91. Epub 2008 Oct 21 doi: 10.1007/s00701-008-0014-6. PMID: 18936876
Newly defined central nervous system neoplasms.
Gyure KA
Am J Clin Pathol 2005 Jun;123 Suppl:S3-12. doi: 10.1309/PCU3FC92CMUW68VA. PMID: 16100866
The WHO classification of tumors of the nervous system.
Kleihues P, Louis DN, Scheithauer BW, Rorke LB, Reifenberger G, Burger PC, Cavenee WK
J Neuropathol Exp Neurol 2002 Mar;61(3):215-25; discussion 226-9. doi: 10.1093/jnen/61.3.215. PMID: 11895036

Clinical prediction guides

Suprasellar chordoid neoplasm with expression of thyroid transcription factor 1: evidence that chordoid glioma of the third ventricle and pituicytoma may form part of a spectrum of lineage-related tumors of the basal forebrain.
Hewer E, Beck J, Kellner-Weldon F, Vajtai I
Hum Pathol 2015 Jul;46(7):1045-9. Epub 2015 Mar 25 doi: 10.1016/j.humpath.2015.03.005. PMID: 25934249
Chordoid glioma of the third ventricle: four cases including one case with papillary features.
Ni HC, Piao YS, Lu DH, Fu YJ, Ma XL, Zhang XJ
Neuropathology 2013 Apr;33(2):134-9. Epub 2012 Jun 21 doi: 10.1111/j.1440-1789.2012.01333.x. PMID: 22716306
Chordoid glioma of the third ventricle: a report of two new cases, with further evidence supporting an ependymal differentiation, and review of the literature.
Pasquier B, Péoc'h M, Morrison AL, Gay E, Pasquier D, Grand S, Sindou M, Kopp N
Am J Surg Pathol 2002 Oct;26(10):1330-42. doi: 10.1097/00000478-200210000-00010. PMID: 12360048
The WHO classification of tumors of the nervous system.
Kleihues P, Louis DN, Scheithauer BW, Rorke LB, Reifenberger G, Burger PC, Cavenee WK
J Neuropathol Exp Neurol 2002 Mar;61(3):215-25; discussion 226-9. doi: 10.1093/jnen/61.3.215. PMID: 11895036
Chordoid glioma of the third ventricle: an ultrastructural study of three cases with a histogenetic hypothesis.
Cenacchi G, Roncaroli F, Cerasoli S, Ficarra G, Merli GA, Giangaspero F
Am J Surg Pathol 2001 Mar;25(3):401-5. doi: 10.1097/00000478-200103000-00016. PMID: 11224612

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