Dysembryoplastic neuroepithelial tumor of the cerebellum and brainstem

Case report

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✓ The authors present a case in which dysembryoplastic neuroepithelial tumors (DNETs) occurred in the cerebellum and brainstem of a 44-year-old woman. A magnetic resonance image of the brain revealed multiple cystic lesions in the right cerebellar hemisphere, vermis, tonsil, and brainstem. Partial removal of the tumors was performed. There were gray multinodular gelatinous lesions on the cerebellar hemisphere. Histologically, the tumors exhibited areas of multiple microcystic nodules in the cerebellar white matter, which were composed of oligodendroglia-like cells (OLCs), astrocytes, and neurons. There were multiple, variable nodules in the lesions, lined by OLCs. The adjacent cerebellar cortex displayed dysplastic features. Reduction of granule neurons and dislocation of Purkinje cells into the molecular layer were observed. The pathological profile of this patient agrees with that described by Daumas-Duport, et al., as a “dysembryoplastic neuroepithelial tumor.”

Article Information

Address reprint requests to: Kenta Fujimoto, M.D., Institute for Neuroradiology, University Hospital Zurich, Frauenklinikstrasse 10, 8091 Zurich, Switzerland.

© AANS, except where prohibited by US copyright law.

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Figures

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    Magnetic resonance images of the brain revealing multiple cystic lesions in the right cerebellar hemisphere, vermis, tonsil, and brainstem. Sagittal image illustrates swelling of the cerebellar tonsil. Left: Axial T1-weighted image. Right: Sagittal T2-weighted image.

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    Macroscopic photograph (A) and photomicrographs (B–E) demonstrating the histopathological findings in this case. H & E. A: Photograph showing the subcortical location of the lesion, including its constituent nodules. B: Alveolar structure with small neuronal cells, astrocytes, and OLCs. There are multiple nodules that contain a mucoid substance and are lined by OLCs. Original magnification × 33. C: Neurons intermixed with glial elements. Original magnification × 66. D: Specific glioneuronal elements. Original magnification × 66. E: Adjacent cerebellar cortex with dysplastic features. A Purkinje cell is seen within the molecular layer. Original magnification × 66.

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    Magnetic resonance image obtained at the 2-year follow-up examination, demonstrating no regrowth of residual tumor.

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