Cortical Tubers

Grossly, the tubers appear as nodule-like structures that result from focal broadening and firmness of the convolutions. Some are slightly elevated above the cortical surface, whereas others display a central dimple. Their sizes vary from a few millimeters to 2 to 3 cm, and their number may reach 30 to 40. They are randomly distributed in the cerebral hemispheres, less frequently in the cerebellum (Figs. 13.27 and 13.28).

Histologically, the normal cortical architecture is replaced by dysplastic neurons and glial cells. The neurons display abnormal shapes and orientation; they contain variable amounts of Nissl substance and, occasionally, neurofibrillary tangles. The astrocytes are large, often bizarrely gigantic, and may form clusters. Dense fibrillary astrocytosis and calcifications are common (Fig. 13.29).

figure 13.28

Tuberous sclerosis in a 15-year-old girl. A. Right cerebral hemisphere displays multiple, sharply circumscribed, firm tubers. Some are elevated above the cortical surface and have a central dimple. B. The left hemisphere displays abnormally oriented small and broad convolutions.

Cortical Tubers

figure 13.29

Tuberous sclerosis. Histology: A. Frontal lobe stained for myelin shows myelin loss beneath the cortical tubers. Within the tubers, the cortical cytoarchitecture is disorganized. It consists of (B) large, atypical neurons, and (C) monstrous astrocytes (cresyl violet). The periventricular nodules consist of (D) dense astrocytic fibrosis with numerous calcified capillaries and (E) a subependymal astrocytoma (HE). F. A heterotopic nodule of glial cells and neuron is present in the hemispheric white matter (LFB-CV).

figure 13.29

Tuberous sclerosis. Histology: A. Frontal lobe stained for myelin shows myelin loss beneath the cortical tubers. Within the tubers, the cortical cytoarchitecture is disorganized. It consists of (B) large, atypical neurons, and (C) monstrous astrocytes (cresyl violet). The periventricular nodules consist of (D) dense astrocytic fibrosis with numerous calcified capillaries and (E) a subependymal astrocytoma (HE). F. A heterotopic nodule of glial cells and neuron is present in the hemispheric white matter (LFB-CV).

Cortical Tubers

figure 13.30

Tuberous sclerosis. Hamartoma of the kidney consists of fibrous, adipose, and angiomatous nodules.

figure 13.30

Tuberous sclerosis. Hamartoma of the kidney consists of fibrous, adipose, and angiomatous nodules.

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