CNS & pituitary tumors

Meningeal tumors

Chordoid meningioma


Editorial Board Member: Meaghan Morris, M.D., Ph.D.
Deputy Editor-in-Chief: Chunyu Cai, M.D., Ph.D.
Valeria Barresi, M.D., Ph.D.

Last staff update: 20 October 2022 (update in progress)

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PubMed Search: Chordoid meningioma

Valeria Barresi, M.D., Ph.D.
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Cite this page: Barresi V. Chordoid meningioma. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/cnstumorchordoidmeningioma.html. Accessed December 9th, 2022.
Definition / general
  • Chordoid meningioma is a histological subtype of meningioma characterized by cords or trabeculae of epithelioid (or spindle) cells embedded in a mucin rich stroma and resembling chordoma
  • It is classified as CNS WHO grade 2, regardless of the presence of worrisome histological features (mitoses, spontaneous necrosis, brain invasion)
Essential features
  • Histological subtype of meningioma that is characterized by cords or trabeculae of epithelioid (or spindle) cells embedded in a mucin rich stroma and resembling chordoma
  • CNS WHO grade 2
  • Recurrence risk is associated with subtotal resection, high Ki67 labeling index and atypical histological features (mitoses, brain invasion, spontaneous necrosis, small cell with high N:C ratio, hypercellularity, macronucleoli, sheeting)
  • Pure forms exist but it most commonly features intermingled areas of classical meningioma, with syncytial growth pattern, whorls and pseudoinclusions
  • Immunohistochemistry: EMA+, podoplanin+, possible focal cytokeratin+, S100-, brachyury-, GFAP-
Terminology
  • Chordoid meningioma
ICD coding
  • ICD-O: 9538/1 - chordoid meningioma
  • ICD-11: XH2LS4 - chordoid meningioma
Epidemiology
Pathophysiology
Etiology
Clinical features
Diagnosis
  • Based on imaging (CT, MRI) / biopsy / resection specimen
Radiology description
Radiology images

Contributed by Valeria Barresi, M.D., Ph.D.

T1 weighted sagittal MRI

T1 sagittal contrast enhancement

T2 weighted axial MRI

FLAIR axial MRI

Prognostic factors
Case reports
Treatment
Gross description
Frozen section description
  • Differential diagnosis versus other tumor types: at least focal presence of psammoma bodies, meningothelial whorls or nuclear pseudoinclusions
  • Differential diagnosis versus other meningioma subtypes: cords of cells with eosinophilic cytoplasm in a myxoid background (NMC Case Rep J 2020;7:53)
Frozen section images

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Intraoperative histopathological findings

Microscopic (histologic) description
Microscopic (histologic) images

Contributed by Valeria Barresi, M.D., Ph.D.

Epithelioid cells

Spindle cells

Intermingled typical meningioma


Mitoses

Alcian blue

Podoplanin immunostaining

Cytology description
Cytology images

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Vacuolated cells

Positive stains
Molecular / cytogenetics description
Molecular / cytogenetics images

Images hosted on other servers:

FISH 18p, 22q, 14q, 1p

Sample pathology report
  • Brain, extra-axial convexity mass:
    • Meningioma, subtype chordoid, CNS WHO grade 2 (see comment)
    • Comment: Meningothelial neoplasia showing epithelioid cells with vacuolated, eosinophilic cytoplasm or spindle cells, arranged in cords, within a myxoid matrix.
Differential diagnosis
  • Chordoma:
    • Most commonly arises in the sacrococcygeal region and is usually extradural and bone invasive
    • Can be intracranial at the clivus, have secondary invasion of the dura or rarely may be extradural
    • Podoplanin negative, brachyury positive and showing extensive staining for CK AE1 / AE3, S100 and EMA
  • Chondrosarcoma:
    • Rarely, may be meningeal
    • Positive for S100 and negative for EMA
  • Chordoid glioma:
    • Rare glioma that is typically localized at the third ventricle
    • GFAP positive
  • Metastatic carcinoma:
    • Metastases of carcinomas may be meningeal
    • They show extensive cytokeratin staining, while chordoid meningioma features only focal staining for keratins
Board review style question #1

An extra-axial mass is found at the clivus without bone invasion. Histological examination shows a tumor with eosinophilic epithelioid cells in a myxoid stroma and focal areas with a syncytial growth pattern, whorls and nuclear pseudoinclusions. What is the most likely diagnosis?

  1. Chondrosarcoma
  2. Chordoid meningioma
  3. Chordoma
  4. Metastasis of carcinoma
Board review style answer #1
B. Chordoid meningioma. Chondrosarcoma may be meningeal and enter the differential diagnosis with chordoid meningioma. Chordoma is frequently localized at the clivus, though it is usually bone invasive. Even in the absence of immunohistochemistry, the presence of areas with meningothelial classical features (whorls, syncytial growth pattern, nuclear pseudoinclusions) is strongly suggestive of a meningioma.

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Reference: Chordoid meningioma
Board review style question #2
An extra-axial mass is found in the spine. Histological examination shows a tumor composed of eosinophilic epithelioid cells in a myxoid matrix. At immunohistochemistry, it is EMA+, cytokeratin+ (focally), podoplanin+, S100- and brachyury -. Which is the most likely diagnosis?

  1. Chondrosarcoma
  2. Chordoid glioma
  3. Chordoid meningioma
  4. Chordoma
Board review style answer #2
C. Chordoid meningioma. Chordoma is EMA+ but also extensively positive for CK AE1 / AE3, S100 and brachyury, while it is negative for podoplanin. Chordoid glioma is localized at the third ventricle and is GFAP+. Chondrosarcoma is S100+ and EMA-.

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Reference: Chordoid meningioma
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