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A case of cerebellar hemangioblastoma with rhabdoid features

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Abstract

We present an unusual case of cerebellar hemangioblastoma characterized by rhabdoid features. The patient was a 35-year-old Japanese man with occipital neuralgia and exacerbating blurred vision. Magnetic resonance imaging revealed a left posterior cranial fossa tumor, which was isointense on T1-weighted images and hyperintense on T2-weighted images with marked homogeneous enhancement. Histology of the surgically resected tumor showed cellular-type hemangioblastoma with extensive proliferation of rhabdoid cells Immunohistochemistry analysis showed tumor cells positive for inhibin A, CD56, vimentin, INI-1, and vascular endothelial growth factor; negative for PAX8, CD10, epithelial membrane antigen, cytokeratin, (AE1/3), alpha-smooth muscle actin and D2-40; and had focal positivity for glial fibrillary acidic protein and S100. The Ki-67 labeling index was <1 %. Ultrastructural analysis revealed large lipid droplets and abundant intracellular accumulation of intermediate filaments. Based on these findings, the diagnosis was hemangioblastoma with focal rhabdoid features. After a 14-month follow-up, there was no evidence of recurrence. This is the first report of hemangioblastoma with rhabdoid features in the central nervous system. In addition, we discuss the possible pathogenesis.

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Authors and Affiliations

  1. Department of Diagnostic Pathology, Kobe University Graduate School of Medicine, 7-5-2, Kusunoki-cho, Chuo-ku, Kobe, 6500017, Japan

    Ayako Tomono, Shigeo Hara & Tomoo Itoh

  2. Department of Gastrointestinal Surgery, Kobe University Graduate School of Medicine, Kobe, Japan

    Ayako Tomono

  3. Department of Pathology, Hyogo Cancer Center, Akashi, Japan

    Takanori Hirose

Authors
  1. Ayako Tomono
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  2. Shigeo Hara
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  3. Takanori Hirose
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  4. Tomoo Itoh
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Correspondence to Shigeo Hara.

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Tomono, A., Hara, S., Hirose, T. et al. A case of cerebellar hemangioblastoma with rhabdoid features. Brain Tumor Pathol 32, 145–150 (2015). https://doi.org/10.1007/s10014-014-0193-1

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  • DOI: https://doi.org/10.1007/s10014-014-0193-1

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