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We describe the case of 47-year-old man with a cystic, melanotic temporal lobe astrocytoma who had a history of complex partial seizures. The tumor mass was made up of two histologically different regions: one consisted of spindle-shaped and pleomorphic cells often with foamy or vacuolated cytoplasm, while the other consisted of fairly uniform spindle-shaped cells, many of which contained dark-brown intracytoplasmic pigment. Desmoplasia was also noted in the latter region of the tumor. No features suggestive of malignancy, such as mitotic figures, necrotic foci or endothelial vascular proliferation, were observed throughout the tumor. Immunohistochemically, the tumor cells in both regions were positive for glial fibrillary acidic protein. Ultrastructural examination of the pigmented region showed the presence of melanosomal melanin in the tumor cells. Apart from the partial pigmentation, the entire histological picture resembled a pleomorphic xanthoastrocytoma. To our knowledge, only two cases of similar melanotic astrocytic tumors have been described previously. Interestingly, the astrocytic tumors in both of these patients were also clinically associated with epilepsy, were located in the temporal lobe, and were histologically benign.

In a girl with mannosidosis, who died at 3 1/2 years of age, histopathological and ultrastructural changes in the nervous system are described. A widespread neuronal storage evidenced by ballooning of the neuronal perikarya is the salient histological feature and the occurrence of electron-lucent storage vacuoloes in neurons, astrocytes, endothelial cells and pericytes is the most striking ultrastructural feature of mannosidosis in the nervous system. By virtue of the deficiency of acidic alpha-mannosidases A and B, the accumulation of mannose-containing oligosaccharides in tissues and the occurrence of storage vacuoles in various cells, mannoisidosis is similar to various neuronal storage diseases associated with lysosomal enzyme deficiencies. In mannosidosis, the storage vacuoles in the neural and visceral tissues are alike with little variation in details and contain chiefly loosely dispersed, finely reticulogranular material. The storage vacuoles in neurons in mannosidosis are, therefore, distinct from those in neurons in other lysosomal storage disease such as Pompe's disease, various lipidoses and mucopolysaccharidoses. However, they resemble closely the storage vacuoles in neurons in fucosidosis and those in liver cells in various mucopolysaccharidoses.