Angiocentric glioma

Definition

An indolent supratentorial glioma showing a perivascualr distribution of tumorus cells and associated with epilepsy.

Clinical features

Patient present with drug-resistant long-term focal epilepsy, usually commencing in childhood.

Radiology

On fluid-attenuated inversion recovery (FLAIR) images, the tumours are solid, circumscribed, hyperintense, non-enhancing; they are cortical, extending into subcortical white matter.

On MRI, T1 weighted images show cortical rim-like hyperintensities. Stalk-like extensions to the ventricles on T2-weighted MRI images and FLAIR are said to be characteristic.

Histopathology

Bipolar spindle cells are arranged around small and medium-size blood vessels, circumferentially, radially or longitudinally. The cells have elongated nuclei with fine chromatin and lack conspicuous nucleoli. Most cases also show small nests of round/epithelioid cells. There may be scattered incorporated neurons. Necrosis is absent and mitotic activity low.

Immunohistochemistry

	GFAP	8/81
	Vimentin	8/81
	S100B, polyclonal	8/81
	EMA	8/8(mainly dot-like)1
	Podoplanin	8/8(sometimes dot-like)1
	EGFR	4/8(weak)1
	CD99	equivocal1
	b-A4-amyloid	0/81
	CD34	0/81
	CD117	0/81
	Microtubule-associated protein 2	0/81
	Neuronal nuclear antigen	0/81
	Neurofilament	0/81
	Oligodendrocyte transcription factor 2	0/81
	p53	0/81
	Reelin	0/81
	Synaptophysin	0/81
	a-synuclein	0/81
	hyperphosphorylated tau	0/81
	CD45	positive in microglial processes
	CD68	positive in microglial processes
	CD8	diffuse T-cells
	Ki-67 proliferation index	<= 5%

Ultrastructure

There are intracellular ciliated lumina with microvilli and zona adherents contacts. Other cases show astrocyte-like intermediate filaments.

Differential diagnosis

Other low-grade gliomas

Management

Surgery

Prognosis

These tumours are usually indolent, although fatalities have occurred.

References

1 Preusser M, Hoischen A, Novak K, et al. Angiocentric glioma: report of clinico-pathologic and genetic findings in 8 cases. Am J Surg Pathol 2007; 31:1709-18

This page last revised 1.1.2008