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malignant peripheral nerve sheath tumor (MPNST)

usually malignant neurofibroma (neurofibroscarcoma), malignant schwannomas being extremely rare. Sarcomatous transformation, particularly of plexiform neurofibromas in neurofibromatosis type 1, can occur. Microscopic pathology: - anaplastic spindle cells - high cellularity - high mitotic activity - histologic subtypes 1) epitheliod MPNST 2) MPNST with epithelial or mesenchymal differentiation 3) melanotic MPNST Immunohistochemistry: - S100: +, less so than schwannoma - CD34: variable - leu 7: +

Interactions

disease interactions

General

nerve sheath tumor soft tissue sarcoma (STS)

References

WHO International Histological Classification of Tumors. Histological Typing of Tumours of the Central Nervous System. Kleihues et al. Springer-Verlag 2nd ed. 1993

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