OBJECTIVETo study the clinicopathologic features of subependymal giant cell astrocytoma.

METHODSThe clinical and pathologic characteristics of 18 cases of subependymal giant cell astrocytoma were retrospectively analyzed.

RESULTSAmongst the 18 cases studied, there was a male predominance (male-to-female ratio = 2:1). The age of patients ranged from 7 to 54 years (mean age = 18.2 years). The tumor often occurred in the lateral ventricles (16/18, 88.9%). Most patients presented with headache and vomiting (11/18, 61.1%), followed by visual disturbance (3/18, 16.7%). Eleven patients (61.1%) had clinical features of tuberous sclerosis, usually in the form of facial angiofibroma (8/18, 44.4%). Computerized tomography was performed in 10 cases, in which 7 cases were of high density and 5 cases showed contrast enhancement. MRI revealed isointense mass lesion on T1WI (7/11, 63.6%), highly intense lesion on T2WI (10/11, 90.9%) and contrast enhancement in some cases (9/11, 81.8%). Four patients had follow-up information available and all of them were alive from 1 to 5 years (mean = 3.5 years). Histologically, there were bundles of spindle cells mixed with clusters of gemistocytes and ganglion-like cells. The spindle cells showed immunoreactivity for glial fibrillary acidic protein (18/18, 100%), while the gemistocytes and ganglion-like cells expressed synaptophysin (14/18, 77.8%). Most of the cases (16/18, 88.9%) had MIB-1 index

CONCLUSIONSSubependymal giant cell astrocytoma is a benign brain tumor with distinctive histopathologic features. The tumor typically affects children and young adults. It is associated with a favorable clinical outcome, especially if completely excised.