Papillary glioneuronal tumor: a new variant of mixed neuronal-glial neoplasm.

Journal Article

We describe the clinicopathologic features of nine cases of a unique papillary glioneuronal tumor (PGNT) exhibiting astrocytic as well as extensive and varied neuronal differentiation. The four male and five female patients studied ranged in age from 11 to 52 years (mean 27.7 years). They either presented with mild neurologic symptoms or were asymptomatic. Magnetic resonance imaging showed demarcated cystic, 1.5-cm to 7-cm contrast-enhancing masses; five involved the temporal lobe, two the parietal, and two the frontal. All but one were totally resected. No recurrence was noted despite a follow-up period of 3 years. Two microscopic components were evident: 1) compact pseudopapillae composed of hyalinized vessels covered by a single layer of glial fibrillary acid protein (GFAP)-positive astrocytes and 2) synaptophysin-positive neuronal cells of varying size, including neurocytes, ganglioid cells, and ganglion cells within neuropil. Immunostains for chromogranin-A were negative, as was in situ hybridization for chromogranin-A mRNA. Ultrastructurally, neuronal cells featured microtubule-containing processes and aberrant synaptic terminals, but dense core granules were rare. Overall, cellularity was moderate and atypia was minimal. No mitotic activity or necrosis was noted. The proportions of the two components varied, but essential morphologic findings were identical in all cases. In that the clinical, radiographic, and morphologic characteristics of PGNT are distinctive, it appears to represent a previously undescribed form of mixed neuronal-glial tumor of the central nervous system.

Full Text

Duke Authors

Cited Authors

  • Komori, T; Scheithauer, BW; Anthony, DC; Rosenblum, MK; McLendon, RE; Scott, RM; Okazaki, H; Kobayashi, M

Published Date

  • October 1998

Published In

Volume / Issue

  • 22 / 10

Start / End Page

  • 1171 - 1183

PubMed ID

  • 9777979

International Standard Serial Number (ISSN)

  • 0147-5185

Language

  • eng

Conference Location

  • United States