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Epithelioid malignant peripheral nerve sheath tumor involving maxillary sinus

We present a case of epithelioid malignant peripheral nerve sheath tumor (MPNST) located in the maxillary sinus region in a young man. The clinical history was short, but at admission the neoplastic infiltration was so extensive that only diagnostic biopsy was performed. The patient received palliative treatment and died 6 months later. Histologically, the neoplasm had a predominant epithelioid component. Neoplastic tissue was vimentin, S‐100, Cam 5.2 and neuron‐specific enolase positive. P53 protein reaction was found in 6% of the cells and the proliferation index assessed with Ki‐67 was 52%. An appropriate immunohistochemical  panel  was  essential  for  the  final  diagnosis  of this epithelioid malignant tumor, with the location rather unusual for MPNST.