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Nerve sheath myxoma with bidirectional schwannomatous and perineural differentiation.

A case of nerve sheath myxoma occurring in occipital region in 70-yr-old woman is presented. The tumor showed typical lobular and myxoid morphology. Immunohistochemically, it showed unusual coexpression of Schwann cell markers S100 protein and GFAP with perineural cell markers EMA and claudin-1. CD34+ fibroblast-like cells were scarce, and nerve axons were not found in the tumor. Clinical pathology and histogenesis of the lesion are discussed.