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恶性周围神经鞘膜瘤（malignant peripheral nerve sheath tumor，MPNST）常转移至肺，胸膜转移罕见。本文报道1例MPNST胸膜转移患者。患者青年男性，因“气促伴干咳1周”就诊。既往恶性周围神经鞘膜瘤病史。内科胸腔镜胸膜活检病理提示短梭形细胞增生，胸膜病理切片免疫组织化学染色S-100（+）、SOX-10（+）、Ki-67（+）阳性指数20%、H3K27Me3（-），诊断为MPNST胸膜转移。

Malignant peripheral nerve sheath tumor (MPNST) frequently metastasizes to the lungs, although pleural metastasis is rare. This article reported a case of pleural metastasis of MPNST. The patient was a young man who presented with 1 week of shortness of breath with dry cough. He had a history of malignant peripheral nerve sheath tumor. The patient was diagnosed with MPNST pleural metastasis after a thoracoscopic pleural biopsy, which revealed short spindle cell hyperplasia, immunohistochemical staining for S-100(+), SOX-10(+), Ki-67(+) with a positive index of 20%, and H3K27Me3(-) in the pleural pathology.