CNS tumor

Other gliomas

Chordoid glioma of the third ventricle

Topic Completed: 1 March 2013

Minor changes: 22 February 2021

Copyright: 2002-2021,, Inc.

PubMed Search: Chordoid glioma of the third ventricle

Eman Abdelzaher, M.D., Ph.D.
Page views in 2020: 1,269
Page views in 2021 to date: 1,330
Cite this page: Abdelzaher E. Chordoid glioma of the third ventricle. website. Accessed November 27th, 2021.
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
  • "Glioma" since GFAP+
  • Uncommon ( < 50 cases reported)
  • Usually middle aged women (median age 45 years, 63% female)
  • Low grade neoplasm arising in third ventricle hypothalamic region
  • Often attached to hypothalamic and suprasellar structures (63%)
  • 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
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
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
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