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Intracranial angiomatoid fibrous histiocytoma with rhabdoid features: a mimic of rhabdoid meningioma

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Abstract

Angiomatoid fibrous histiocytoma (AFH) is an uncommon soft-tissue neoplasm that arises mostly in the extremities of young people and generally carries a good prognosis. Intracranial location is unusual and frequently associated with myxoid change. EWSR1 gene fusions with members of the CREB family (CREB1, ATF1, and CREM) are well-established events in AFH. These fusions have also been described in other neoplasms including intracranial myxoid mesenchymal tumor, and it is still uncertain whether the latter is a distinct entity or if it represents a myxoid variant of AFH. Here, we describe a rare falcine AFH presenting in a 50-year-old woman. The most striking feature of this tumor was its diffuse rhabdoid morphology with focal high mitotic activity, raising the consideration of rhabdoid meningioma (WHO grade III). The tumor cells were moderately positive for EMA and negative for progesterone receptor and SSTR2 prompting additional studies. Desmin was strongly positive and CD99 showed membranous immunoreactivity. BAP1, INI-1, and BRG1 expressions were retained. Next-generation sequencing analysis demonstrated an EWSR1–ATF1 gene fusion, supporting the diagnosis of an unusual rhabdoid variant of AFH. After gross total resection of this tumor, the patient remains free of disease 5 months after the surgery without additional treatment.

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Acknowledgements

The authors render thanks to Julie A. Vrana, Ph.D. and Kurt S. Willkomm for their technical expertise in the immunohistochemical and molecular data for this manuscript.

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Authors and Affiliations

  1. Department of Laboratory Medicine and Pathology, Mayo Clinic Rochester, 200 1st St SW, Rochester, MN, 55905, USA

    M. Adelita Vizcaino, Caterina Giannini, Benjamin R. Kipp, Karen Fritchie & Rachael Vaubel

  2. Department of Pathology, Sparrow Hospital, Lansing, 1215 E Michigan Ave, Lansing, MI, 48912, USA

    Howard T. Chang

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  1. M. Adelita Vizcaino
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  2. Caterina Giannini
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Correspondence to Rachael Vaubel.

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Vizcaino, M.A., Giannini, C., Chang, H.T. et al. Intracranial angiomatoid fibrous histiocytoma with rhabdoid features: a mimic of rhabdoid meningioma. Brain Tumor Pathol 38, 138–144 (2021). https://doi.org/10.1007/s10014-020-00389-5

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  • DOI: https://doi.org/10.1007/s10014-020-00389-5

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