Ganglioneuroblastoma of the conus: A rare and aggressive tumor

摘要

Sir, The term ganglioneuroblastoma' is used for tumors that histologically consist of small round immature tumor cells in all stages of neuronal differentiation with scattered immature ganglion cells. These tumors are grouped under 'primitive neuroectodermal tumors' (PNETs): (1) 'peripheral' or 'pPNET'- those that arise from the sympathetic nervous system and (2) 'central' or 'cPNET' - those that arise within the central nervous system (CNS). Although both may be histologically similar, pPNET and cPNET have different clinical, immunochemical and genetic profiles. These tumors have only rarely been found in the CNS. Even rarer is the occurrence of a primary intraspinal ganglioneuroblastoma. Of the reported cases of intramedullary ganglion cell tumors, there was only one case of ganglioneuroblastoma, while all others were cases of gangliogliomas, ganglioneuromas, gangliocytomas, neurocytomas or mixed-type tumors. The occurrence of an unusual and aggressive intramedullary ganglioneuroblastoma of the conus in a young girl, which is so far an unreported entity, prompted us to present this case.