The 2007 WHO Classification of Nervous System Tumors: newly recognized members of the mixed glioneuronal group - PubMed (original) (raw)

The 2007 WHO Classification of Nervous System Tumors: newly recognized members of the mixed glioneuronal group

Marc K Rosenblum. Brain Pathol. 2007 Jul.

Abstract

The clinical and pathologic features of two glioneuronal neoplasms newly incorporated in the 2007 revision of the WHO classification of nervous system tumors are reviewed. These are the papillary glioneuronal tumor and the rosette-forming glioneuronal tumor of the fourth ventricle.

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Figures

Figure 1

Figure 1

As is often the case, the solid component of this papillary glioneuronal tumor is contrast‐enhancing on post‐gadolinium T1‐weighted MR imaging. Note its projection into a sizable cyst.

Figure 2

Figure 2

Histologic and immunophenotypic features of papillary glioneuronal tumor. A. Papillary gliovascular structures (left) abruptly give way to solid components containing oligodendrocyte‐like clear‐cell neurocytic elements. B. Small, cuboidal cells of uniform appearance cover hyalinized fibrovascular cores in papillary regions. C. Cytoplasmic immunolabeling for glial fibrillary acidic protein attests to the glial nature of papillary elements. D. Non‐papillary components include small round cells, many of oligodendrocyte‐like appearance but representing neurocytes, as well as larger neurons (arrow). E. Neuronal elements of intermediate type (“ganglioid” cells) exhibit vesicular nuclei and distinct nucleoli, but little rimming cytoplasm. Note myxoid change. F. Intense synaptophysin labeling is evidenced in intermediate neuronal elements, while cells surrounding vascular papillae (left) are non‐reactive. Deeply eosinophilic cells of minigemistocytic or coarsely granulated appearance may be found adjacent to papillary structures (G), or admixed with small, clear‐cell neurocytic elements (H).

Figure 3

Figure 3

Filling the fourth ventricle, this rosette‐forming glioneuronal tumor exhibits linear contrast‐enhancement on post‐gadolinium T1‐weighted MR imaging.

Figure 4

Figure 4

Histologic and immunophenotypic features of rosette‐forming glioneuronal tumor (RGNT). A. Diminutive rosettes often seem to float in tumoral clefts or cavities (right) that border solid astrocytic components. Perivascular pseudorosetting is also seen (left). B. True Homer Wright‐like rosettes consist of delicate fibrillar cores rimmed by small, monomorphous, rounded nuclei. C. Perivascular pseudorosettes assume columnar configurations when sectioned along vascular axes. Myxoid and microcystic alterations are apparent. D. Rosettes consistently manifest granular synaptophysin immunoreactivity of their cores. E. Glial components of the RGNT are usually dominated by spindled astroglial cells that may achieve moderate cell density and are immunoreactive for glial fibrillary acidic protein (GFAP) (F). G. Small oligodendrocyte‐like cells may also populate glial regions and are non‐reactive for GFAP and neuron‐associated antigens. H. Complex microvascular proliferation is a feature of some RGNTs.

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