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Malignant Peripheral Nerve Sheath Tumor of the Skin in Various Presentation
Hui-Wen Cheng , Chia-Yi Yang , Kai-Yam Ng , Fen-Yi Sung , Ting-An Chang
Dermatol Sinica 22 : 79-87, 2004

Malignant peripheral nerve sheath tumor(MPNST) was a spindle cell sarcoma, which could arise from nerve tissue, neurofibroma or showing nerve sheath differentiation. Cutaneous MPNST was especially rare compared with the tumors of deep soft tissue. Microscopically, all tumor cells revealed irregular contours, high cellularity and pleomorphism, located mainly in the dermis. These tumor cells were all with wavy or comma-shaped nuclei arranged in storiform or fascicular pattern. Hyperchromatism and atypical mitosis were quite prominent. Immunohistochemical studies demonstrated the evidence of neural differentiation. Clinically, an insidiously growing skin tumor which showed pleomorphic spindle cells proliferation on histopathology study, MPNST of the skin should be considered as one of the differential diagnosis. Herein, we reported 3 cases of cutaneous MPNST with unique presentation of gradually enlarging skin tumors, which were considered of sporadic type, associated with neurofibroma and neurofibromatosis type 1 respectively.

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