Objective: To study the clinicopathologic features of subependymal giant cell astrocytoma.
Methods: The clinical and pathologic characteristics of 18 cases of subependymal giant cell astrocytoma were retrospectively analyzed.
Results: Amongst 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 <or= 1%.
Conclusions: Subependymal 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.