Multifocal Neurocytoma/Gangliocytoma with Extensive Leptomeningeal Dissemination in the Brain and Spinal Cord

Abstract

This report describes an unusual neuronal tumor detected at the autopsy of a 17-year-old boy. The tumor showed multifocal parenchymal involvement with extensive leptomeningeal dissemination. The intraparenchymal lesions were small and located mainly in the subpial region of the cerebrum, cerebellum and spinal cord. Leptomeningeal dissemination was particularly pronounced at the base of the brain and around the spinal cord and presumably took place during the relatively long clinical course. The tumor was composed of small round cells and ganglion-cell-like cells. Only neuronal differentiation, as represented by immunostaining with antisynaptophysin antibody and the presence of dense-core vesicles in the cytoplasm, was evident in both types of cells. The small round cells appeared to exhibit the features of small, relatively mature neurons rather than those of neuroblasts. Moreover, our results suggested maturation from small round cells to ganglion-cell-like cells. The tumor appears to be related to gangliogliomas or dysembryoplastic neuroepithelial tumors, and we have chosen the term neurocytoma/gangliocytoma for the unusual lesion.

Diffuse Leptomeningeal Glioneuronal Tumors: A New Entity?

“…These four tumors are characterized by similar histological appearance, mixed immunohistochemical profile combining neuronal/glial markers and widespread tumor cell invasion of the leptomeninges without evidence of a primary intraparenchymal mass. We believe that similar cases have been already published in the literature, but otherwise called diffuse leptomeningeal oligodendroglioma (1–3, 5–8, 18) or neurocytoma/gangliocytoma (21). Because of the unique similar clinical and neuroradiological characteristics, we propose to consider this group of neoplasms as a new possible distinct pathological and clinical entity in the group of glioneuronal tumors.…”

Gamma knife radiosurgery of recurrent central neurocytomas: a preliminary report

“…18 20 24 In line with the general characteristics of central neurocytomas, our patients were young adults, the onset of symptoms was abrupt, and all patients became symptomatic with signs of increased intracranial pressure 414 15 17 25 26 The tumour locations in our series were mainly both lateral ventricles with extension to the third ventricle in one patient, such as described in previous reports 2…”

Central neurocytoma: a clinico-pathological study of five cases

“…8,9 M ore recently a multifocal neurocytic tum our with leptom eningeal dissem ination has been reported. 10 The m ean age of 27 years of our cases is similar to that reported in previous series. The sym ptom s of raised intracranial pressure are related to the hydrocephalus.…”