Papillary glioneuronal tumorと Rosette-forming glioneuronal tumorの病理組織像

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  • 小森 隆司
    財団法人東京都医学研究機構東京都神経科学総合研究所 臨床神経病理研究部門

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  • Pathological findings of papillary glioneuronal tumor and rosette-forming glioneuronal tumor
  • Papillary glioneuronal tumor ト Rosette forming glioneuronal tumor ノ ビョウリ ソシキゾウ

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Glioneuronal tumors （GNT） are mixed glial and neuronal tumors of the central nervous system and are essentially benign neoplasms. In the WHO 2007 Classification, the category of GNT contains 12 entities, three of which, extraventricular neurocytoma, papillary glioneuronal tumor （PGNT）, rosette-forming glioneuronal tumor （RGNT）, were new additions to the current edition. PGNT is comprised of two microscopic components: 1） compact pseudopapillae composed of hyalinized vessels covered by a single layer of GFAPpositive astrocytes; 2） synaptophysin-positive neuronal cells of varying size, including neurocytic, ganglioid and ganglion cells within neuropil. RGNT is also comprised of two microscopic components: 1） synaptophysinpositive neurocytes forming neurocytic and/or perivascular pseudorosettes in a fibrillary, partly microcystic matrix; 2） the component resembling pilocytic astrocytoma, which consisted of fibrillated spindle cells with oval nuclei associated with occasional Rosenthal fibers,and oligodendrocyte-like cells （OLC）. In that their clinical, radiographic, and morphologic characteristics are distinctive, PGNT and RGNT represent heretofore undescribed forms of GNT. Recent development of highly sensitive immunohistochemistly certainly aids a daily practice but also arouse unexpected controversies in an identification of neuronal phenotype as well as tumor entities of GNT. These include the presence of neuronal immuno-phenotype in malignant gliomas. The presence of OLC in PGNT as well as RGNT further suggest their derivation from neuronal-oligodendrocytic progenitor cells.

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