CNS tumor
Neuronal and mixed neuronal - glial tumors
Papillary glioneuronal tumor (PGNT)

Author: Eman Abdelzaher, M.D., Ph.D. (see Authors page)

Revised: 31 August 2017, last major update May 2014

Copyright: (c) 2002-2017, PathologyOutlines.com, Inc.

PubMed Search: Papillary glioneuronal tumor [title]

Cite this page: Abdelzaher, E. Papillary glioneuronal tumor (PGNT). PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/cnstumorpapillaryglioneuronal.html. Accessed December 11th, 2017.
Definition / general
  • Rare, low grade biphasic neoplasm characterized by pseudopapillae lined by glial cells with interpapillary neuronal elements
  • Favorable outcome suggests that it corresponds to WHO grade I
  • First described by Komori et al. (Am J Surg Pathol 1998;22:1171)
Epidemiology
  • Age: young adults
  • Sex: no gender predilection
Sites
  • Cerebral hemisphere
  • > Temporal lobe
Radiology description
  • Supratentorial, discrete, contrast enhancing
  • Often cystic with occasional cyst mural nodule pattern
Radiology images

Images hosted on other servers:

Discrete cystic mass

Heterogeneously enhancing mass in pineal gland

Case reports
Treatment
  • Complete surgical resection
  • Favorable clinical outcome
Microscopic (histologic) description
  • Biphasic pattern with glial and neuronal components
  • Glial component: small uniform glial cells line pseudopapillae with hyalinized vascular cores; no atypia or mitosis
  • Neuronal component: interpapillary zone is occupied by neurocytes with uniform nuclei and occasional perinuclear halos; occasionally ganglion and ganglioid cells are admixed
  • Vascular proliferation and necrosis are exceptional
  • The surrounding brain tissue shows gliosis
Microscopic (histologic) images

Images hosted on other servers:

Papillary glioneuronal tumor

H&E, synaptophysin, Ki67, GFAP, MAP

GFAP, synaptophysin

Cytology description
  • Nonspecific, uniform cells
Immunohistochemistry
Electron microscopy description
  • Three cell types: astrocytic, neuronal, glioneuronal progenitor cells
Differential diagnosis