Neurosurgery notes/Tumours/Gliomas, Glioneuronal Tumors, and Neuronal Tumors/Circumscribed astrocytic gliomas/Chordoid glioma of the third ventricle

Chordoid glioma of the third ventricle

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Circumscribed astrocytic gliomas

Definition

  • Essential:
    • A glial neoplasm with chordoid features located In the anterior 3rd ventricle
  • Desirable:
    • Nuclear thyroid transcription factor 1 (TTF1) immunopositivity
    • PRKCA p.D463H mutation or DNA methylation profile aligning with chordoid glioma
  • WHO CNS 2016 Old: A slow-growing, non-invasive glial tumour located in the third ventricle, histologically characterized by clusters and cords of epithelioid tumour cells expressing GFAP, within a variably mucinous stroma typically containing a lymphoplasmacytic infiltrate.

Numbers

  • Rare: 80 cases reported
  • 46 years
  • Male: Female = 1:2

CNS WHO grading

  • Grade 2

Localisation

  • Occupy the anterior portion of the third ventricle

Origin

  • Arise in the region of the lamina terminalis in the ventral wall of the third ventricle
  • Ependymal cell: because CG3rd has microvilli and hemidesmosomes-like structure

Clinical features

  • Obstructive hydrocephalus, including headache, nausea, vomiting, and ataxia
  • Hypothalamic compression: pituitary dysfunction hypothyroidism, amenorrhoea, and diabetes insipidus),
  • Chiasmatic compression: visual field disturbance
  • Personality changes/psychiatric abnormalities

Radiology

  • Hypothyroidism, amenorrhoea, and diabetes insipidus),well-circumscribed ovoid masses within the anterior third ventricle
  • T1-isointense to brain and show strong, homogeneous contrast enhancement
  • Most tumours are continuous with the hypothalamus and some appear to have an intrinsic anterior hypothalamic component, suggesting a potential site of origin
 
A close-up of a mri scan AI-generated content may be incorrect.

Histopathology

  • Micro
      • Solid neoplasms, most often composed of clusters and cords of epithelioid tumour cells within a variably mucinous stroma that typically contains a lymphoplasmacytic infiltrate
      • Other less common histological pattern;
        • A solid pattern with sheets of polygonal epithelioid tumour cells without mucinous stroma,
        • A fusiform pattern with groups of spindle shaped cells among loose collagen, and
        • A fibrosing pattern with abundant fibrosis.
          • More in older patients
      • Mitoses are absent in most tumours; when present, they are rare (< 1 mitosis per 10 high-power fields)
      • Consistent with their radiographical appearance, the tumours are architecturally solid and show little tendency to infiltrate surrounding brain structures.
      • Reactive astrocytes, Rosenthal fibres, and often chronic inflammatory cells including lymphocytes, plasma cells, and Russell bodies are seen in adjacent nonneoplastic tissue
      • + GFAP. TTF1Vimentin, CD34, EGFR, Merlin
      • Variable S100, EMA, cytokeratin
      • - Neuronal and neuroendocrine markers (e.g. synaptophysin, neurofilaments, and chromogranin-A)
      • Ki-67 proliferation index is low, with values of 0-1.5%
      Fig. 4.03 Chordoid glicrna of the third ventricle. A Histologicaly, tLmours are characterized by cohesive clusters of epithelioid cells with abundant pink cytoplasm and a bubbly, bluish, mucin-rich stoma. B At higher magnifcatlon, nuclei are oval, mc&rate in size, and bland, and have dispersed chromatim Mitotc activity and nuclear atypia are absent. C In almost every Instance, tLmour cells also fom solid arrangements of either nests or linear arrays. Lymphoplasmacytic Infiltrate present in nearly all Chordoid gliomas, and Russell bodies can be identifed (E, anows). F The border between Chordoid gliama and adjacent brain is well defined, with litte evidence of tumour infiltration, often 'Mth chronic inflammation (arrcw) and Rosenthal fibres in the neighbouring brain.
      Choroid glioma of the third ventricle. A Histologically, tumours are characterized by cohesive clusters of epithelioid cells with abundant pink cytoplasm and a bubbly, bluish, mucin-rich stroma. B At higher magnification, nuclei are oval, moderate in size and bland, and have dispersed chromatin; mitotic activity and nuclear atypia are absent. C In almost every instance, tumour cells also form solid arrangements of either nests or linear arrays. D,E Lymphoplasmacytic infiltrates present in nearly all Choroid gliomas and Russell bodies can be identified (E, arrows). F The border between Choroid glioma and adjacent brain is well defined, with little evidence of tumour infiltration, often with chronic inflammation (arrow) and Rosenthal fibres in the neighbouring brain.

Differential diagnosis

  • Chordoid meningiomas usually contain small foci of classic meningioma with whorl formation and psammoma bodies; they are also immunopositive for EMA, but negative for GFAP and CD34 {2235}.
  • Chordomas strongly express cytokeratins and brachyury, but lack immunoreactivity for GFAP and CD34.

Prognosis

  • Gross total resection is the treatment of choice and can result in long-term recurrence-free survival
    • The tumours’ location within the third ventricle and their attachment to hypothalamic and suprasellar structures often make complete resection impossible.
  • Postoperative tumour enlargement has been noted in half of all patients who undergo subtotal resection.
    • 20% of the patients died in the perioperative period or from tumour regrowth