Spongioneuroblastoma and Tuberous Sclerosis

In a series of remarkable publications, Globus (1, 2, 3, 4, 5, 6) investigated the histology of primary cerebral neoplasms of neuro-ectodermal origin composed of both neural and glial elements, and established the diagnostic groupings of glioneuromas and spongioneuroblastomas. The glioneuroma is characterized, histologically, by well differentiated nerve cells exhibiting small cell bodies, a restricted amount of Nissl substance and paucity of processes, distributed among fairly dense masses of equally differentiated neuroglial elements, showing small cell bodies and short delicate processes. The consists of cell types of a linage similar to those of the glioneuroma, but of a more primitive character, early stages of maturation of neurons being observed among spongioblastic elements. Previous to Globus' publications, scanty attention was given to these types of tumors which had been collected as pathologic curiosities under various names: ganglioneuroma, neuroglioma, ganglioblastoma, ganglioglioneuroma, and ganglioglioma. In his studies on the spongioneuroblastoma, Globus (1, 2, 3, 4) stressed the fact that almost all tumors of this type are found in association with lesions of either fully developed or abortive forms of tubero-sclerosis. He coined the term spongioneuroblastoma disseminatum to denote the pathological process of tubero-sclerosis. It is the purpose of this paper to describe a new instance of occurring in tubero-sclerosis. Unusual features of the case were the extraordinary extension of the tumor involving the whole hemisphere, the striking abundance of neuronal cells within the tumor, and the apparently neoplastic transformation of the cellular elements of the cerebral cortex.