CNS & pituitary tumors

Gliomas, glioneuronal tumors and neuronal tumors

Neuronal and mixed neuronal-glial tumors

Papillary glioneuronal tumor


Editorial Board Member: P.J. Cimino, M.D., Ph.D.
Deputy Editor-in-Chief: Chunyu Cai, M.D., Ph.D.
Eman Abdelzaher, M.D., Ph.D.

Last author update: 4 June 2024
Last staff update: 4 June 2024

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PubMed Search: Papillary glioneuronal tumor

Eman Abdelzaher, M.D., Ph.D.
Page views in 2024 to date: 1,005
Cite this page: Abdelzaher E. Papillary glioneuronal tumor. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/cnstumorpapillaryglioneuronal.html. Accessed July 13th, 2024.
Definition / general
  • Low grade glioneuronal tumor with a biphasic pattern composed of pseudopapillary glial structures and interpapillary neuronal components; also characterized by PRKCA gene fusion (mainly SLC44A1::PRKCA fusion)
  • Corresponds to WHO grade 1
Essential features
  • Rare, low grade glioneuronal neoplasm with biphasic histological and immunophenotypic pattern characterized by pseudopapillae lined by GFAP positive glial cells with interpapillary synaptophysin positive neuronal elements (Acta Neurochir (Wien) 2016;158:695, AJNR Am J Neuroradiol 2022;43:1080, Acta Neuropathol Commun 2015;3:85)
  • First described by Komori et al. (Am J Surg Pathol 1998;22:1171)
  • Papillary glioneuronal tumor (PGNT) is characterized by PRKCA gene fusion (mainly SLC44A1::PRKCA fusion) and a distinctive methylation profile
  • Supratentorial, well delineated and often cystic tumors with predilection for young adults
  • Favorable prognosis with gross total resection
Terminology
  • Not recommended by WHO: pseudopapillary ganglioneurocytoma, pseudopapillary neurocytoma with glial differentiation
ICD coding
  • ICD-O: 9509/1 - papillary glioneuronal tumor
  • ICD-11: 2A00.21 & XH3XU4 - mixed neuronal glial tumors & papillary glioneuronal tumor
Epidemiology
Sites
Pathophysiology
Etiology
  • Unknown
Clinical features
Diagnosis
  • Neuroimaging: magnetic resonance imaging (MRI) and computed tomography (CT)
  • Biopsy: diagnosis should be heavily weighted toward molecular findings because morphological analyses frequently result in mistyping (Acta Neuropathol 2019;137:837)
  • WHO diagnostic criteria
    • WHO essential diagnostic criteria
      • Biphasic histological and immunophenotypic pattern with pseudopapillary glial lining and interpapillary neuronal components
      • PRKCA gene fusion (mostly SLC44A1::PRKCA)
      • For unresolved lesions: methylation profile of papillary glioneuronal tumor
    • WHO desirable diagnostic criteria
      • Well delineated, solid and cystic tumor
Radiology description
Radiology images

Contributed by Eman Abdelzaher, M.D., Ph.D.
MRI, axial T1

MRI, axial T1

MRI, sagittal T1

MRI, sagittal T1

MRI, axial T1 with contrast

MRI, axial T1 with contrast

MRI, sagittal T1 with contrast

MRI, sagittal T1 with contrast

MRI, axial FLAIR

MRI, axial FLAIR

MRI, axial T2

MRI, axial T2



Images hosted on other servers:
Contrast enhancement and ventricular association

Left temporal lobe mass, MRI

Left temporal lobe mass, MRI

Contrast enhancement and ventricular association

Prognostic factors
Case reports
Treatment
Clinical images

Images hosted on other servers:
Right occipital lobe tumor

Right occipital lobe tumor

Left frontal lobe tumor

Left frontal lobe tumor

Gross description
  • Well circumscribed, solid and / or cystic lesions
  • Calcification and hemorrhage may be observed
Microscopic (histologic) description
Microscopic (histologic) images

Contributed by Eman Abdelzaher, M.D., Ph.D.
Pseudopapillary structures Pseudopapillary structures Pseudopapillary structures

Pseudopapillary structures

Gliovascular structures and intervening neuronal cells

Gliovascular structures and intervening neuronal cells

Hyalinized glial pseudopapillae with interpapillary neuronal component Biphasic pattern, low power

Hyalinized glial pseudopapillae with interpapillary neuronal component


Hyalinized gliovascular structures with intervening neuronal cells Hyalinized gliovascular structures with intervening neuronal cells

Hyalinized gliovascular structures with intervening neuronal cells

Hyalinized glial pseudopapillae with neuronal component

Hyalinized glial pseudopapillae with neuronal component

Pseudopapillary glial structures with neuronal component

Pseudopapillary glial structures with neuronal component

Hyalinized vascular cores and neuronal component

Hyalinized vascular cores and neuronal component

Glial and neuronal components

Glial and neuronal components


Glial pseudopapillae and neuronal component

Glial pseudopapillae and neuronal component

Neuronal cells with perinuclear halos

Neuronal cells with perinuclear halos

Glial pseudopapillae and neuronal component

Glial pseudopapillae and neuronal component

Glial and neuronal components

Glial and neuronal components

GFAP GFAP

GFAP


GFAP GFAP

GFAP

Synaptophysin Synaptophysin

Synaptophysin

Synaptophysin Synaptophysin

Synaptophysin

Cytology description
Cytology images

Images hosted on other servers:
Papillaroid fragments; loosely cohesive clusters

Papillaroid fragments; loosely cohesive clusters

Smear preparation

Smear preparation


Positive stains
Electron microscopy description
Molecular / cytogenetics description
Molecular / cytogenetics images

Images hosted on other servers:
9;17 translocation; interphase FISH studies

9;17 translocation; interphase FISH studies

Interphase FISH analysis

Interphase FISH analysis

RT PCR

RT PCR


Videos

Case 4, papillary glioneuronal tumor

Sample pathology report
  • Left temporal lobe mass lesion, gross total resection:
    • Papillary glioneuronal tumor, CNS WHO grade 1
    • Molecular genetics: PRKCA gene fusion (SLC44A1::PRKCA)
Differential diagnosis
Board review style question #1

A 26 year old man presented with seizures. Magnetic resonance imaging (MRI) showed a left temporal lobe mass lesion with an enhancing cyst mural nodule pattern. Gross total resection of the tumor was done and the histopathological features of the tumor are shown in the image above. Which of the following genetic abnormalities is the hallmark of this tumor?

  1. 1p/19q codeletion
  2. KIAA1549::BRAF fusion
  3. MN1 alteration
  4. PRKCA gene fusion
Board review style answer #1
D. PRKCA gene fusion (mainly SLC44A1::PRKCA) is the hallmark of papillary glioneuronal tumor (PGNT). Answer A is incorrect because 1p/19q codeletion is characteristic of oligodendroglioma. Answer B is incorrect because KIAA1549::BRAF fusion is characteristic of pilocytic astrocytoma. Answer C is incorrect because MN1 alteration is the hallmark of astroblastoma.

Comment Here

Reference: Papillary glioneuronal tumor
Board review style question #2
Which of the following is the most distinctive histological feature of papillary glioneuronal tumor?

  1. Biphasic with compact elongated piloid cells alternating with loosely textured spongy areas
  2. Biphasic with pseudopapillary gliovascular structures associated with intervening neuronal cells ranging from neurocytes to ganglion cells
  3. Intracortical lesion with a distinctive nodular pattern with a myxoid background and floating neurons
  4. Neurocytic rosettes or perivascular pseudorosettes with synaptophysin+ neuropil
Board review style answer #2
B. Biphasic with pseudopapillary gliovascular structures associated with intervening neuronal cells ranging from neurocytes to ganglion cells. Papillary glioneuronal tumor is histologically characterized by a biphasic pattern composed of pseudopapillary glial structures and interpapillary neuronal components. Answer A is incorrect because pilocytic astrocytoma is histologically characterized by a biphasic pattern with compact elongated piloid cells alternating with loosely textured spongy areas. Answer C is incorrect because dysembryoplastic neuroepithelial tumor is histologically characterized by being an intracortical lesion with distinctive nodular pattern with a myxoid background and floating neurons. Answer D is incorrect because neurocytic rosettes or perivascular pseudorosettes with syanptophysin+ neuropil are the histological hallmarks of rosette forming glioneuronal tumor.

Comment Here

Reference: Papillary glioneuronal tumor
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