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Fourth ventricle neurocytoma with lipomatous and ependymal differentiation

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Abstract

We report the case of a 4-year-old girl with a fourth ventricle tumor diagnosed as a cerebellar liponeurocytoma which recurred, showing ependymal differentiation, 14 months after surgery. Magnetic resonance imaging at initial presentation revealed a large mass in the fourth ventricle, and histology showed a neoplasm characterized by a combination of well-differentiated neurocytes and cells resembling adipocytes. The tumor recurrence was histologically identical to the original tumor in some regions, but with fewer adipose-like cells, while other areas presented an endocrine architecture with oligo-like or pleiomorphic cells, and rosette-like arrangements of tumoral cells were seen around the thin vessels, with features similar to cellular ependymoma. The cells in the liponeurocytoma areas expressed synaptophysin, chromogranin A, and epithelial membrane antigen. Glial fibrillary acidic protein was expressed in some dispersed tumoral cells, in lipidized tumoral cells, and in reactive astrocytes. Cytokeratin was focally expressed in the ependymal region of the recurrence. The immunophenotype of our case, with glial, ependymal, and neuronal or neuroendocrine markers, suggest a neurocytoma with lipomatous and ependymal differentiation. This tumor resembled those derived from circumventricular organs. Its localization in the area postrema region led us to hypothesize that it may be derived from this circumventricular organ.

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Acknowledgments

The authors thank T. Barkas for linguistic help. This work was supported by INSERM and the Association for Cancer Research (ARC 3277).

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Authors and Affiliations

  1. Hôpital Neurologique, BP Lyon Montchat, , 69394, Lyon Cedex 03, France

    Anne Jouvet

  2. Laboratoire d’Anatomie Pathologique, Hôpital Necker Enfants Malades, Paris, France

    Arielle Lellouch-Tubiana, Nathalie Boddaert & Michel Zerah

  3. INSERM U433, Faculté de Médecine RTH Laennec, 69372, Lyon Cedex 08, France

    Jacques Champier & Michelle Fèvre-Montange

Authors
  1. Anne Jouvet
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  2. Arielle Lellouch-Tubiana
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  3. Nathalie Boddaert
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  4. Michel Zerah
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  5. Jacques Champier
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  6. Michelle Fèvre-Montange
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Correspondence to Michelle Fèvre-Montange.

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The first two authors contributed equally to this work

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Jouvet, A., Lellouch-Tubiana, A., Boddaert, N. et al. Fourth ventricle neurocytoma with lipomatous and ependymal differentiation. Acta Neuropathol 109, 346–351 (2005). https://doi.org/10.1007/s00401-004-0963-7

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  • DOI: https://doi.org/10.1007/s00401-004-0963-7

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