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Spinal glioneuronal tumor with neuropil‐like islands and meningeal dissemination: histopathological and radiological study of a pediatric case
Authors:Pietro Luigi Poliani  Domenico Sperli  Sergio Valentini  Antonio Armentano  Luisa Bercich  Maria Fausta Bonetti  Giuseppe Corriero  Massimo Brisigotti  Aldo Quattrone  Pier Luigi Lanza
Affiliation:1. Department of Pathology I, University of Brescia, Brescia, Departments of;2. Pediatrics,;3. Neuroradiology and;4. Neurosurgery, Azienda Ospedaliera di Cosenza, Cosenza,;5. Department of Pathology, Ospedale Infermi, Rimini, and;6. Institute of Neurological Sciences, National Research Council, Mangone, Cosenza, Italy
Abstract:
Cerebral and spinal location of glioneuronal tumors have been recently described as a novel type of primary CNS neoplasia. A distinctive rare form of glioneuronal tumors with neuropil‐like islands (GTNI) have been reported to occur in the adult cerebrum, whereas spinal GTNI localization is extremely rare. In the present report we describe a case of a 15‐month‐old child with a spinal GTNI of the cervical region and meningeal dissemination. Histologically the tumor was composed of round, small neurocytic‐like cells arranged around eosinophilic neuropil cores and embedded in a diffuse fibrillar glial component forming prominent “rosetted” neuropil islands displaying strong immunoreactivity for neuronal markers. Cerebral GTNI shows abundant glial components not rarely exhibiting anaplastic features that justify their inclusion within the group of diffuse astrocytomas. In contrast, including our case, spinal GTNI do not show histological evidence of anaplastic features and exhibits a significant neuronal component that may imply considering these lesions in a separate group. Nevertheless, due to their exceptional rarity, the natural history of these lesions is not yet fully understood, but spinal GTNI seems to have an unfavorable clinical course despite their benign histopathological features, which must be taken into account for appropriate treatment and follow‐up of the patient.
Keywords:glioneuronal tumor  neuronal rosettes  neuropil islands  pediatric  spinal cord
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