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Pages: 363-369
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Subependymal Giant Cell Astrocytoma : Epidemiological, clinical, histological and immunohistochemical characteristics :Three cases presentation

Author: Nawal hammas, My Youssef Alaoui Lamrani, Boubacar Efared, mustapha Maaroufi, Elfaiz Mohamed Chaoui, Hind El Fatemi, Laila chbani

Category: JMSR Pathology


Background: Subependymal giant cell astrocytoma (SEGA) is a rare benign tumor, which typically occurs in the lateral ventricle. Its histogenesis is poorly understood.

Cases presentation: We reviewed the clinical, histological and immunohistochemistrical characteristics of three cases of subependymal giant cell astrocytoma. The age ranged between 6 years old and 10 years old. Two of patients were male. All patients show symptoms in relation to intracranial hypertension with seizures in one case. All the tumors were located in the lateral ventricle. One patient showed tuberous sclerosis complex signs (subcortical tubers). A tumor resection was performed in all cases and microscopic examination revealed a tumor composed of ganglion-like cells, spindle cells, gémistocytic-like cells and multinucleated cells. Immunohistochemistry showed focal Glial fibrillary acid protein (GFAP) positivity and neurofilament, synaptophysin and S100 protein negativity in all cases. Ki-67 labeling index ranged from 1% to 10%.

Conclusion: Astrocytoma subependymal giant cell is a rare benign tumor that affects children and young adults. Its association with tuberous sclerosis complex is well known. Most patients tend to present with convulsions and symptoms related to intracranial hypertension. Radiologically, CT scan and resonance magnetic imaging do not show specific signs. Microscopically, this tumor is conventionally composed of a proliferation of three cell types: ganglion-like cells; fibrillar spindle cells; and gemistocytic-like cells. Immunohistochemistry reveals that both glial and neuronal proteins are expressed. If the histological diagnosis of this tumor is enough easy, the main problem remains in its histogenesis. SEGA is a low-grade tumor with excellent prognosis if surgically removed.

Keywords: Subependymal giant cell astrocytoma, brain neoplasm, tuberous sclerosis complex, histogenesis, immunohistochemistry.