Problems in the nosology of desmoplastic tumors of childhood

Several types of central nervous system tumors primarily consisting of a combination of astrocytes and fibrocollagen and occurring most commonly in infancy have been separately introduced as new diagnostic entities. The names applied to these tumors have included 'gliofibroma', 'desmoplastic cerebral astrocytoma', and 'desmoplastic infantile ganglioglioma', a group containing ganglion cells as well as the astrocytes and mesenchymal tissue. We studied a gliofibroma arising in the fourth ventricle of a 6-month-old infant and three examples of desmoplastic cerebral astrocytoma in infants utilizing routine histological and immunocytochemical methods, and at the ultrastructural level in two tumors. In view that the desmoplastic cerebral astrocytomas contain poorly differentiated neuroepithelial cells and rarely, a ganglion cell, we suggest that gliofibroma and desmoplastic astrocytoma are basically the same entity and that the desmoplastic infantile ganglioglioma is a variant containing primitive or mature ganglion cells.