A case of a pregnant woman suffering from von Recklinghausen disease (neurofibromatosis) is presented. After the delivery a tumor from the left arm that was recently enlarged was ressected. The neoplasm proved to be a plexiform neurofibroma with malignant transformation to malignant schwannoma. We present the morphological and immunohistochemical findings of this rare entity, we discuss the problems of differential diagnosis, and we review the recent literature.

Key words: von Recklinghausen disease, Neurofibromatosis, Malignant Schwannoma, Pregnancy.

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