CNS & pituitary tumors
Gliomas, glioneuronal tumors, and neuronal tumors
Neuronal and mixed neuronal-glial tumors
Papillary glioneuronal tumor
Author: Eman Abdelzaher, M.D., Ph.D.
Last author update: 1 May 2014
Last staff update: 29 February 2024 (update in progress)
Copyright: 2002-2024, PathologyOutlines.com, Inc.
PubMed Search: Papillary glioneuronal tumor [title]
Table of Contents
Definition / general | Epidemiology | Sites | Radiology description | Radiology images | Case reports | Treatment | Microscopic (histologic) description | Cytology description | Immunohistochemistry & special stains | Electron microscopy description | Differential diagnosis | Additional referencesCite this page: Abdelzaher E. Papillary glioneuronal tumor. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/cnstumorpapillaryglioneuronal.html. Accessed April 19th, 2024.
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 1
- 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:
Heterogeneously enhancing mass in pineal gland
Case reports
- 14 year old girl with papillary glioneuronal tumor (Arq Neuropsiquiatr 2004;62:869)
- 18 year old man with papillary glioneuronal tumor (Arch Pathol Lab Med 2000;124:1820)
- 58 year old man with malignant papillary glioneuronal tumor of the pineal gland (Am J Case Rep 2013;14:164)
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
Cytology description
- Nonspecific, uniform cells
Immunohistochemistry & special stains
- Glial elements: GFAP+
- Neuronal elements: synaptophysin+
- Low proliferation index (Ki67 is 1 - 2%)
Electron microscopy description
- Three cell types: astrocytic, neuronal, glioneuronal progenitor cells
Differential diagnosis
- Astroblastoma: dot-like EMA reactivity, synaptophysin negative
- Clear cell ependymoma: synaptophysin negative
- DNET: intracortical, specific glioneuronal elements with intervening floating neurons
- Extraventriular neurocytoma: no pseudopapillae lined by glial cells
- Oligodendroglioma: nonenhancing, diffusely infiltrative
- Pilocytic astrocytoma: biphasic piloid and spongy areas
- RGNT: 4th ventricle, neurocytic rosettes with synaptophysin positive cores
Additional references
