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Angiosarcoma arising in schwannoma of cerebellopontine angle and later associating with meningioma in a patient with neurofibromatosis type 2

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Abstract

Here, we describe an extremely rare case of angiosarcoma arising in schwannoma of the cerebellopontine angle and later associating with meningioma in a patient with neurofibromatosis type 2. A 33-year-old disabled Japanese man with right drop foot after surgery for an unspecified tumor demonstrated multiple tumors, suspected to be schwannoma, in the bilateral cerebellopontine angles, the cervical and lumbar spinal cord, and on the right nuchal skin. Also present were several tumors in the medulla and thoracic spinal cord suspected to be ependymoma or astrocytoma. The patient was diagnosed with neurofibromatosis type 2 according to the diagnostic criteria by the U.S. National Institutes of Health. The bilateral tumors in the cerebellopontine angle were resected to reduce symptoms and brain stem compression. Histopathological analysis revealed angiosarcoma arising in schwannoma of the bilateral tumors, and angiosarcoma was proportionally larger in the right tumor than in the left. At age 36, the patient underwent a second resection of the regrown tumor in the left cerebellopontine angle, and histopathology demonstrated mixed angiosarcoma and meningioma. That angiosarcoma arises in schwannoma is a pathogenesis within the realm of conjecture, especially that the phenomenon of mixed meningioma and angiosarcoma has not been reported to date.

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The authors have no grant for this research and no conflict of interest to declare.

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Authors and Affiliations

  1. Department of Diagnostic Pathology, Kobe University Hospital, 7-5-2 Kusunoki-cho, Chuo-ku, Kobe-shi, Hyogo, 650-0017, Japan

    Yasuhiro Sakai, Ayako Tomono, Fumi Kawakami & Tomoo Itoh

  2. Department of Pathology for Regional Communication, Kobe University Hospital, Hyogo, Japan

    Takanori Hirose

  3. Department of Diagnostic Pathology, Nara Medical University Hospital, Nara, Japan

    Tokiko Nakai & Chiho Ohbayashi

  4. Department of Neurosurgery, Kobe University Hospital, Hyogo, Japan

    Takashi Mizowaki, Kazuhiro Tanaka & Eiji Kohmura

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  1. Yasuhiro Sakai
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  2. Takanori Hirose
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  3. Ayako Tomono
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  4. Fumi Kawakami
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  5. Tokiko Nakai
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  7. Takashi Mizowaki
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  10. Tomoo Itoh
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Correspondence to Yasuhiro Sakai.

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Sakai, Y., Hirose, T., Tomono, A. et al. Angiosarcoma arising in schwannoma of cerebellopontine angle and later associating with meningioma in a patient with neurofibromatosis type 2. Brain Tumor Pathol 31, 293–298 (2014). https://doi.org/10.1007/s10014-014-0180-6

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  • DOI: https://doi.org/10.1007/s10014-014-0180-6

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