• World Neurosurg · Apr 2017

    Case Reports

    Unusual Intracerebral Presentation of a Myxoid Neurofibroma: A Case Report.

    • da Costa Marcos Devanir Silva MDS Department of Neurosurgery, Federal University of Sao Paulo, Sao Paulo-SP, Brazil. Electronic address: marcoscostaneuro@gmail.com., Karina Hoshino, João Norberto Stavale, Oreste Paulo Lanzoni, Sergio Cavalheiro, and Manoel Antonio Paiva Neto.
    • Department of Neurosurgery, Federal University of Sao Paulo, Sao Paulo-SP, Brazil. Electronic address: marcoscostaneuro@gmail.com.
    • World Neurosurg. 2017 Apr 1; 100: 713.e5-713.e8.

    BackgroundNeurofibromas are benign nerve sheath tumors that usually affect peripheral nerves and are related to neurofibromatosis type 1; however, they have not been described as a cause of intraparenchymal brain tumor.Case DescriptionWe report a case of intracranial myxoid neurofibroma in a 19-year-old female patient manifested as an intense and progressive cephalea, followed by nausea, vomiting, photophobia, and phonophobia. Computed tomography and magnetic resonance imaging showed an extant, expansive left frontoparietal parafalcine/parasagittal tumor. Histopathologic examination determined S-100 protein and CD34 positivity, as well as sparse expression of Ki67 protein, and indicated Schwann cells with characteristic wavy nuclei and intraneural fibroblasts in a myxoid background. Together, these observations characterized the tumor as myxoid neurofibroma. The tumor was excised, and the patient recovered without deficits and with no signs of recurrence after 6 years of follow-up.ConclusionsThis is a novel presentation of a myxoid neurofibroma. The tumorigenesis mechanisms are likely complex and possibly involve the differentiation of Schwann cells present in adrenergic autonomic nerves in the subarachnoid arterial branches or in trigeminal nerves present in the meningeal convexity.Copyright © 2017 Elsevier Inc. All rights reserved.

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