Subependymal giant cell astrocytoma — a clinicopathological study of 23 cases with special emphasis on histogenesis

Abstract

Subependymal giant cell astrocytomas (SEGAs) are relatively rare tumors but occur commonly in the setting of the familial syndrome of tuberous sclerosis complex (TSC). In view of its varied morphology, i.e. resemblance to astrocytic and ganglion cells, its histogenesis remains controversial. We studied 23 cases of SEGA, 19 from our own institute and 4 from NIMHANS, Bangalore. These 19 cases of SEGAs were collected over a period of 23 years (1979 to 2001), and accounted for 0.16% of intracranial tumors and 0.51% of all gliomas reported at our center. The majority of patients presented with visual disturbances (19/23, 82.6%) in the form of decreased vision (60.8%) and blindness (21.7%), generalized tonic clonic seizures (43.4%) and focal motor seizures (4.37%). Age ranged from 4 to 37 years (mean 13.2 years) with male predominance (M:F 2.2:1), and the duration of symptoms varied from 1 month to 96 months (mean 17.2 months). Lateral ventricular involvement was the most common site (91.3%), followed by the third ventricle (8.6%). Nine patients (39.1%) had stigmata of tuberous sclerosis (6 at the time of diagnosis and 3 in the follow-up period). Two patients died due to surgical complications, while the rest were alive and well in the follow-up period ranging from 3 to 264 months (mean 37.1 months). Two patients experienced recurrences, one two years and another 22 years after surgery. Microscopic examination showed varied histology consisting of sweeping bundles of spindle cells, gemistocyte and ganglion-like cells with interspersed inflammatory cell component. The inflammatory cell component on special staining turned out to be an admixture of mast cells and T lymphocytes. Six cases showed areas of necrosis and/or mitosis, but were not indicative of aggressive nature of this tumor. Immunoreactivity for GFAP, NF, S-100, NSE and synaptophysin indicates that this is a hybrid tumor with glial and neuronal differentiation. None of the tumors was immunopositive for HMB-45. The significance of the presence of T lymphocytes and mast cells is not clear. It could be related to tumor immunology and may indicate a favorable prognosis.

Tuberous sclerosis complex and sphenoid meningioma

“…This occurrence suggests that neoplasms arising from hematoma and may behave differently from those CNS tumors that arise apparently de novo 6 . Subependymal giant cell astrocytomas are slowly growing tumors corresponding to WHO grade I 6-7 , accounted for 0.16% of intracranial tumors and 0.51% of all gliomas reported and shows varied histology consisting of sweeping bundles of spindle cells, gemistocyte and ganglion-like cells with interspersed inflammatory cell component 6 . Immunoreactivity for GFAP, NF, S-100, NSE and synaptophysin indicates that this is a hybrid tumor with glial and neuronal differentiation 6,7 .…”

“…Those are grouped by abnormal glial and neuronal proliferation, migration and cortical organization. Non-neoplastic forms include focal cortical dysplasia and tuberous sclerosis, resemblance to astrocytes and ganglion cells, its histogenesis remains controversial 6,7 . Gliomas of the CNS associated with tuberous sclerosis have been well documented [6][7][8] .…”

“…Non-neoplastic forms include focal cortical dysplasia and tuberous sclerosis, resemblance to astrocytes and ganglion cells, its histogenesis remains controversial 6,7 . Gliomas of the CNS associated with tuberous sclerosis have been well documented [6][7][8] . This occurrence suggests that neoplasms arising from hematoma and may behave differently from those CNS tumors that arise apparently de novo 6 .…”

“…Gliomas of the CNS associated with tuberous sclerosis have been well documented [6][7][8] . This occurrence suggests that neoplasms arising from hematoma and may behave differently from those CNS tumors that arise apparently de novo 6 . Subependymal giant cell astrocytomas are slowly growing tumors corresponding to WHO grade I 6-7 , accounted for 0.16% of intracranial tumors and 0.51% of all gliomas reported and shows varied histology consisting of sweeping bundles of spindle cells, gemistocyte and ganglion-like cells with interspersed inflammatory cell component 6 .…”

“…Subependymal giant cell astrocytomas are slowly growing tumors corresponding to WHO grade I 6-7 , accounted for 0.16% of intracranial tumors and 0.51% of all gliomas reported and shows varied histology consisting of sweeping bundles of spindle cells, gemistocyte and ganglion-like cells with interspersed inflammatory cell component 6 . Immunoreactivity for GFAP, NF, S-100, NSE and synaptophysin indicates that this is a hybrid tumor with glial and neuronal differentiation 6,7 . Although giant cell astrocytoma may develop from these subependymal nodules, tumor formation is very rare in the cortical nodules 7 .…”