Chordoid meningioma

Definition

A rare type of grade II meningioma characterised by the presence of vacuolated trabeculae of cells in a myxoid background.

Synonyms

Myxoid meningioma, vacuolated meningioma.

Epidemiology

Chordoid meningiomas occur across a wide age range, including children.

Clinical features

There is an association with systemic disease including Castleman's disease.

Histopathology

The chordoid component consists of cells with vacuolated cytoplasm and well-demarcated cell borders. The background is fibrous or myxoid. The extent of the chordoid component is variable. It merges with areas of typical meningioma. There may be a lymphoplasmacytic inflammatory component.

Immunohistochemistry, comparing morphologically similar tumours

	Chordoid meningioma	Chordoid glioma	Skeletal myxoid chondrosarcoma	Extraskeletal myxoid chondrosarcoma	Low-grade chondrosarcoma	Chordoma	Enchondroma
D2-40	8/101, 2/21a	3/41	6/61	0/161	21/221	0/181	26/271
S100	4/101 , 10%1a	4/41	6/61	13/161, 48%(of 146 cases)1a	22/221	17/181	27/271
AE1/AE3, Cam5.2	2/101, 20%1a	4/41	0/61	6/161, 3%(of 92 cases)1a	0/221	18/181	0/271
EMA	9/101 , 80%1a	4/41	0/61	4/161, 10%(of 97 cases)1a	0/221	17/181	0/271
Brachyury	0/101	0/41	0/61	0/161	0/221	18/181	0/271
GFAP	0/101, negative1a	4/41	0/61	0/161	0/221	0/181	0/271
Vimentin	positive1a
CD57	4/51a
Progesterone receptor	3/31a
CEA	0/41a
CD34	0/41a
Desmin	0/41a
SMA	0/31a
Synaptophysin	0/31a
Chromogranin	0/21a
CD56	0/11a
CD99	0/11a
Calponin	0/11a
34bE12	0/11a
Cytokeratin 14	0/11a
Cytokeratin 19	0/11a
Factor VIII	0/11a
Neurofilament	0/11a
p63	0/11a
Muscle specific actin	0/11a

Differential diagnosis

Chordoma: similar histological appearance, positive for Brachyury, negative for D2-40.
Skeletal myxoid chondrosarcoma
Extraskeletal myxoid chondrosarcoma: negative for D2-40.
Low-grade chondrosarcoma
Enchondroma
Chordoid glioma
Myxopapillary ependymoma
Metastatic carcinoma

Prognosis

There is a high rate of recurrence.

References

1 Sangoi AR, Dulai MS, Beck AH, et al. Distinguishing chordoid meningiomas from their histologic mimics: an immunohistochemical evaluation. Am J Surg Pathol 2009; 33:669-81: 1a meta-analysis of English language publications