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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

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

Recent clinical studies

Etiology

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
Papillary glioneuronal tumor--a rare entity: report of four cases and brief review of literature.
Agarwal S, Sharma MC, Singh G, Suri V, Sarkar C, Garg A, Kumar R, Chandra PS
Childs Nerv Syst 2012 Nov;28(11):1897-904. Epub 2012 Aug 7 doi: 10.1007/s00381-012-1860-3. PMID: 22868530
The expanding family of glioneuronal tumors.
Allende DS, Prayson RA
Adv Anat Pathol 2009 Jan;16(1):33-9. doi: 10.1097/PAP.0b013e3181915e3b. PMID: 19098465
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

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: 35512827Free 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
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
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

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

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

Clinical prediction guides

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
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
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

Recent systematic reviews

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

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