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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

GTR
MeSH
Orphanet

CClinical test, RResearch test, OOMIM, GGeneReviews, VClinVar

CROGVPapillary glioneuronal tumor

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
        Papillary glioneuronal tumor

Follow this link to review classifications for Papillary glioneuronal tumor in Orphanet.

Professional guidelines

PubMed

Treatment and Outcome in 65 Children with Optic Pathway Gliomas.

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

See all (1)

Recent clinical studies

Etiology

Imaging manifestations of papillary glioneuronal tumors.

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: 38649515 Free PMC Article

The landscape of common genetic drivers and DNA methylation in low-grade (epilepsy-associated) neuroepithelial tumors: A review.

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

Papillary glioneuronal tumor (PGNT) exhibits a characteristic methylation profile and fusions involving PRKCA.

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

Papillary glioneuronal tumors: A radiopathologic correlation.

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

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

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Diagnosis

Imaging manifestations of papillary glioneuronal tumors.

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: 38649515 Free PMC Article

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

Recurrent Papillary Glioneuronal Tumor.

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

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

Papillary glioneuronal tumor.

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

See all (44)

Therapy

Glioneuronal tumors of cerebral hemisphere in children: correlation of surgical resection with seizure outcomes and tumor recurrences.

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

Treatment and Outcome in 65 Children with Optic Pathway Gliomas.

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

Aggressive papillary glioneuronal tumor: case report and literature review.

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

See all (3)

Prognosis

Recurrent Papillary Glioneuronal Tumor.

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

Surgical Resection and Cellular Proliferation Index Predict Prognosis for Patients with Papillary Glioneuronal Tumor: Systematic Review and Pooled Analysis.

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

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

Clinicopathologic and neuroradiologic studies of papillary glioneuronal tumors.

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

Papillary glioneuronal tumor: a case report and review of the literature.

Williams SR, Joos BW, Parker JC, Parker JR
Ann Clin Lab Sci 2008 Summer;38(3):287-92. PMID: 18715860

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Clinical prediction guides

Papillary glioneuronal tumor (PGNT) exhibits a characteristic methylation profile and fusions involving PRKCA.

Clinicopathologic and neuroradiologic studies of papillary glioneuronal tumors.

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

SLC44A1-PRKCA fusion in papillary and rosette-forming glioneuronal tumors.

Nagaishi M, Nobusawa S, Matsumura N, Kono F, Ishiuchi S, Abe T, Ebato M, Wang Y, Hyodo A, Yokoo H, Nakazato Y
J Clin Neurosci 2016 Jan;23:73-75. Epub 2015 Aug 7 doi: 10.1016/j.jocn.2015.04.021. PMID: 26260115

Glial tumors with neuronal differentiation.

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

Papillary glioneuronal tumor.

Prayson RA
Arch Pathol Lab Med 2000 Dec;124(12):1820-3. doi: 10.5858/2000-124-1820-PGT. PMID: 11100065

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