CNS tumor
Meningeal tumors
Chordoid meningioma
Topic Completed: 1 January 2015
Minor changes: 24 July 2020
Copyright: 2002-2020, PathologyOutlines.com, Inc.
PubMed Search: Chordoid meningioma [title]
Table of Contents
Definition / general | Sites | Radiology images | Prognostic factors | Case reports | Treatment | Gross description | Microscopic (histologic) description | Cytology description | Cytology images | Positive stains | Negative stains | Differential diagnosis | Additional referencesCite this page: Kresak J, Yachnis A. Chordoid meningioma. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/cnstumorchordoidmeningioma.html. Accessed August 7th, 2020.
Definition / general
- WHO grade II due to tendency to recur
- < 1% of all meningiomas
- Usually adults (mean age 47 years) with no systemic symptoms
- Cases in young adults / pediatric population may be associated with hematologic conditions, such as Castleman disease
Sites
- 80% are supratentorial but also infratentorial, intraventricular, in spinal cord and optic nerve
Radiology images
Images hosted on other servers:
MRI
Enlarged residual tumor
Prognostic factors
- Subtotal resection is associated with earlier time to recurrence (Histopathology 2013;62:1002)
Case reports
- 11 year old boy with intraventricular chordoid meningioma (J Neurosurg Pediatr 2012;10:478)
- 30 year old woman with pyrogenic cytokine interleukin 6 expression by a chordoid meningioma (J Neurosurg 2005;103:555)
- 36 year old woman with primary optic nerve sheath chordoid meningioma (J Clin Neurosci 2010;17:397)
- 38 year old woman with chordoid meningioma, part of a multiple intracranial meningioma (Malays J Med Sci 2013;20:91)
- 39 year old woman with headache (Case of the Week #242)
- 50 year old woman with progressive headaches, unstable gait, short term memory deficit and mood swings (Arch Pathol Lab Med 2004;128:e115)
- Intraventricular chordoid meningioma presenting with Castleman disease (J Neurosurg 2005;102:733)
- Skull base chordoid meningioma (Australas Radiol 2004;48:233)
- Two patients with chordoid meningioma and Castleman syndrome (J Neurooncol 2011;104:395)
Treatment
- Gross total resection when possible
- Postoperative radiation often added for subtotal resections
Gross description
- Dural based
- Well circumscribed
Microscopic (histologic) description
- Called "chordoid" because it resembles a chordoma, with cords or trabeculae of epithelioid cells that may be eosinophilic or clear (resembling physaliferous cells) within a mucin rich or myxoid stroma
- Often traditional meningioma features are intermixed
- Contains a variable lymphoplasmacytic infiltrate
Cytology description
- Cords of polygonal tumor cells with bland nuclei and nuclear pseudoinclusions
- Occasional loose cells with abundant metachromatic pink purple cells without cytoplasmic vacuoles
- Also lymphoplasmacytic infiltrate (Acta Cytol 2004;48:259, Acta Cytol 2004;48:397)
Cytology images
Images hosted on other servers:
Vacuolated cells
Negative stains
Differential diagnosis
- Chondrosarcoma: S100+ (EMA -, CK-)
- Chordoid glioma: usually third ventricle, GFAP positive
- Chordoma: brachyury+, cytokeratin+, EMA+
- Metastatic carcinoma: cytokeratin+
Additional references
