Subependymal giant cell astrocytoma

Magnetic resonance imaging of a subependymal giant cell astrocytoma

Subependymal giant cell astrocytoma in the pathological specimen. The tumor crosses the midline and has caused enlargement of the lateral ventricles .

The Subependymal Riesenzellastrozytom (Engl. Giant subependymal cell astrocytoma , shortly SEGA ) is a rare benign brain tumor which is below the ependyma is growing and especially in the tuberous sclerosis occurs. According to the WHO classification of tumors of the central nervous system , the benign tumor is classified as grade I.

clinic

Subependymal giant cell astrocytomas occur particularly in children and young adults. Since the tumor develops preferentially in the wall of the ventricular system in the vicinity of the Foramina Monroi, the outflow of cerebrospinal fluid is often disturbed as it grows. The tumor usually grows very slowly and shows up on imaging as a circumscribed mass with calcifications and signs of older bleeding. In some cases, the SEGA is the first manifestation of tuberous sclerosis (TSC) and is therefore one of the main diagnostic criteria. However, only 6–16% of all TSC patients develop a SEGA.

Neuropathology

Typical histology of the SEGA with astrocytically differentiated giant cells

The acidic glial fiber protein (GFAP) is heterogeneously expressed in SEGAs (brown staining of the tumor cells)

Histologically, giant astrocytic cells are characteristic of the tumor; however, the morphology of tumor cells can range from polygonal cells with glassy cytoplasm and ganglioid nuclei to spindle-shaped cells in a fibrillar matrix . The tumor cells show a variable expression of GFAP, S-100, synaptophysin and neurofilament.

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