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Papillary glioneuronal tumor

MedGen UID:
457400
Concept ID:
C2985174
Neoplastic Process
Synonyms: Papillary Glioneuronal Tumor; papillary glioneuronal tumor; Papillary Glioneuronal Tumor (WHO Grade 1); Papillary Glioneuronal Tumor (WHO Grade I); papillary glioneuronal tumor (WHO grade I); papillary glioneuronal tumour (WHO grade I); PGNT; Pseudopapillary ganglioglioneurocytoma; pseudopapillary ganglioglioneurocytoma; Pseudopapillary neurocytoma with glial differentiation; pseudopapillary neurocytoma with glial differentiation
SNOMED CT: Papillary glioneuronal tumor (450902001)
 
Monarch Initiative: MONDO:0016735
Orphanet: ORPHA251962

Definition

A rare mixed neuronal-glial tumor characterized by a supratentorial space-occupying lesion in periventricular location, often with prominent cystic change. The histological hallmark of this low-grade neoplasm is its pseudopapillary appearance with a single layer of cuboidal cells around hyalinized blood vessels, associated with sheets or focal collections of neuronal cells. Clinical presentation is variable and non-specific, most frequently with headache and seizures. Prognosis is favorable after complete resection. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPapillary glioneuronal tumor
Follow this link to review classifications for Papillary glioneuronal tumor in Orphanet.

Professional guidelines

PubMed

El Beltagy MA, Reda M, Enayet A, Zaghloul MS, Awad M, Zekri W, Taha H, El-Khateeb N
World Neurosurg 2016 May;89:525-34. Epub 2016 Feb 18 doi: 10.1016/j.wneu.2016.02.042. PMID: 26898488

Recent clinical studies

Etiology

Du X, He Y, Li F, Wang X, Kong X, Ye M, Chen X
Neurosurg Rev 2024 Apr 23;47(1):179. doi: 10.1007/s10143-024-02393-1. PMID: 38649515Free PMC Article
Jesus-Ribeiro J, Rebelo O, Ribeiro IP, Pires LM, Melo JD, Sales F, Santana I, Freire A, Melo JB
Neuropathology 2022 Dec;42(6):467-482. Epub 2022 Jul 17 doi: 10.1111/neup.12846. PMID: 35844095
Hou Y, Pinheiro J, Sahm F, Reuss DE, Schrimpf D, Stichel D, Casalini B, Koelsche C, Sievers P, Wefers AK, Reinhardt A, Ebrahimi A, Fernández-Klett F, Pusch S, Meier J, Schweizer L, Paulus W, Prinz M, Hartmann C, Plate KH, Reifenberger G, Pietsch T, Varlet P, Pagès M, Schüller U, Scheie D, de Stricker K, Frank S, Hench J, Pollo B, Brandner S, Unterberg A, Pfister SM, Jones DTW, Korshunov A, Wick W, Capper D, Blümcke I, von Deimling A, Bertero L
Acta Neuropathol 2019 May;137(5):837-846. Epub 2019 Feb 13 doi: 10.1007/s00401-019-01969-2. PMID: 30759284
Yadav N, Rao S, Saini J, Prasad C, Mahadevan A, Sadashiva N
Eur J Radiol 2017 Dec;97:44-52. Epub 2017 Oct 5 doi: 10.1016/j.ejrad.2017.10.004. PMID: 29153366
Cenacchi G, Giangaspero F
J Neuropathol Exp Neurol 2004 Mar;63(3):185-92. doi: 10.1093/jnen/63.3.185. PMID: 15055442

Diagnosis

Du X, He Y, Li F, Wang X, Kong X, Ye M, Chen X
Neurosurg Rev 2024 Apr 23;47(1):179. doi: 10.1007/s10143-024-02393-1. PMID: 38649515Free PMC Article
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: 35512827Free PMC Article
Goethe EA, Youssef M, Patel AJ, Jalali A, Goodman JC, Mandel JJ
World Neurosurg 2019 Aug;128:127-130. Epub 2019 May 9 doi: 10.1016/j.wneu.2019.04.266. PMID: 31078809
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
Vajtai I, Kappeler A, Lukes A, Arnold M, Lüthy AR, Leibundgut K
Pathol Res Pract 2006;202(2):107-12. Epub 2006 Jan 18 doi: 10.1016/j.prp.2005.11.008. PMID: 16413693

Therapy

Tomita T, Volk JM, Shen W, Pundy T
Childs Nerv Syst 2016 Oct;32(10):1839-48. Epub 2016 Sep 20 doi: 10.1007/s00381-016-3140-0. PMID: 27659827
El Beltagy MA, Reda M, Enayet A, Zaghloul MS, Awad M, Zekri W, Taha H, El-Khateeb N
World Neurosurg 2016 May;89:525-34. Epub 2016 Feb 18 doi: 10.1016/j.wneu.2016.02.042. PMID: 26898488
Newton HB, Dalton J, Ray-Chaudhury A, Gahbauer R, McGregor J
Clin Neuropathol 2008 Sep-Oct;27(5):317-24. doi: 10.5414/npp27317. PMID: 18808063

Prognosis

Goethe EA, Youssef M, Patel AJ, Jalali A, Goodman JC, Mandel JJ
World Neurosurg 2019 Aug;128:127-130. Epub 2019 May 9 doi: 10.1016/j.wneu.2019.04.266. PMID: 31078809
Ahmed AK, Dawood HY, Gerard J, Smith TR
World Neurosurg 2017 Nov;107:534-541. Epub 2017 Aug 18 doi: 10.1016/j.wneu.2017.08.041. PMID: 28823671
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
Zhao RJ, Zhang XL, Chu SG, Zhang M, Kong LF, Wang Y
Acta Neurochir (Wien) 2016 Apr;158(4):695-702. Epub 2016 Feb 22 doi: 10.1007/s00701-016-2744-1. PMID: 26899971
Williams SR, Joos BW, Parker JC, Parker JR
Ann Clin Lab Sci 2008 Summer;38(3):287-92. PMID: 18715860

Clinical prediction guides

Hou Y, Pinheiro J, Sahm F, Reuss DE, Schrimpf D, Stichel D, Casalini B, Koelsche C, Sievers P, Wefers AK, Reinhardt A, Ebrahimi A, Fernández-Klett F, Pusch S, Meier J, Schweizer L, Paulus W, Prinz M, Hartmann C, Plate KH, Reifenberger G, Pietsch T, Varlet P, Pagès M, Schüller U, Scheie D, de Stricker K, Frank S, Hench J, Pollo B, Brandner S, Unterberg A, Pfister SM, Jones DTW, Korshunov A, Wick W, Capper D, Blümcke I, von Deimling A, Bertero L
Acta Neuropathol 2019 May;137(5):837-846. Epub 2019 Feb 13 doi: 10.1007/s00401-019-01969-2. PMID: 30759284
Yadav N, Rao S, Saini J, Prasad C, Mahadevan A, Sadashiva N
Eur J Radiol 2017 Dec;97:44-52. Epub 2017 Oct 5 doi: 10.1016/j.ejrad.2017.10.004. PMID: 29153366
Zhao RJ, Zhang XL, Chu SG, Zhang M, Kong LF, Wang Y
Acta Neurochir (Wien) 2016 Apr;158(4):695-702. Epub 2016 Feb 22 doi: 10.1007/s00701-016-2744-1. PMID: 26899971
Park CK, Phi JH, Park SH
Neurosurg Clin N Am 2015 Jan;26(1):117-38. doi: 10.1016/j.nec.2014.09.006. PMID: 25432191
Prayson RA
Arch Pathol Lab Med 2000 Dec;124(12):1820-3. doi: 10.5858/2000-124-1820-PGT. PMID: 11100065

Recent systematic reviews

Ahmed AK, Dawood HY, Gerard J, Smith TR
World Neurosurg 2017 Nov;107:534-541. Epub 2017 Aug 18 doi: 10.1016/j.wneu.2017.08.041. PMID: 28823671

Supplemental Content

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    Clinical resources

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