CNS tumor
Neuroepithelial tumors of uncertain origin
Chordoid glioma of the third ventricle

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

Revised: 29 August 2017, last major update March 2013

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

PubMed Search: Chordoid glioma of the third ventricle

Cite this page: Abdelzaher, E. Chordoid glioma of the third ventricle. PathologyOutlines.com website. http://pathologyoutlines.com/topic/cnstumorchordoidglioma.html. Accessed September 19th, 2017.
Definition / general
  • Rare, discrete, slow growing glial tumor of third ventricle of adults, characterized by chordoid architecture and myxoid background
  • First described in 1998 (J Neuropathol Exp Neurol 1998;57:283)
  • Cells may resemble ependyma of subcommissural organ, present in dorsocaudal third ventricle during embryonic life, which regresses after birth
Terminology
  • "Glioma" since GFAP+
Epidemiology
  • Uncommon ( < 50 cases reported)
  • Usually middle aged women (median age 45 years, 63% female)
Sites
  • Low grade neoplasm arising in third ventricle hypothalamic region
  • Often attached to hypothalamic and suprasellar structures (63%)
Grading
  • WHO grade II
  • WHO 2000 lists as "glial tumor of uncertain origin"
Radiology description
Radiology images

Images hosted on other servers:

Sagittal T1 weighted MR

Axial CT scan reveals hyperdense mass

Various images

Prognostic factors
Case reports
Treatment
Clinical images

Images hosted on other servers:

Optic chiasm enlargement

Gross description
  • Discrete firm mass adherent to wall of third ventricle
Microscopic (histologic) description
  • Chordoma-like features
  • Clusters and cords of epithelioid cells in mucinous matrix
  • Cells have abundant eosinophilic cytoplasm and round / oval nuclei with indistinct nucleoli
  • Also lymphoplasmacytic infiltrates, Russell bodies, discrete border with peritumoral piloid gliosis
  • May have chondroid metaplasia or papillary formations
  • No / rare mitotic figures, no vascular proliferation, no necrosis
Microscopic (histologic) images

Images hosted on other servers:

Various images

H&E, Alcian blue, GFAP, CD34, EMA and CK

H&E and GFAP

Positive stains
Negative stains
Electron microscopy description
  • Ependymal differentiation
  • Apical pole has microvilli and basal pole has hemidesmosome-like structures connecting cell membranes to basal lamina
  • Also submicroscopic cell body zonation and secretory granules (Am J Surg Pathol 2001;25:401)
Differential diagnosis
  • Chordoid meningioma: whorls, psammoma bodies and nuclear pseudoinclusions, EMA+, GFAP-, well formed desmosomes, 22q-
  • Chordoma: infiltrates bone, physaliphorous cells, EMA+, diffusely keratin+, S100+, GFAP-, mitochondria rough ER complexes