Hybrid Peripheral Nerve Sheath Tumors, Including a Malignant Variant in Type 1 Neurofibromatosis

Plexiform neurofibroma Neurofibromatosis type I
DOI: 10.1097/dad.0b013e31827e2917 Publication Date: 2013-05-15T16:39:56Z
ABSTRACT
The authors report a small case series of hybrid nerve sheath tumors occurring in the setting type 1 neurofibromatosis. Four lesions were benign and consisted plexiform neurofibromas with considerable areas perineuriomatous differentiation patients In these lesions, biphasic (Schwannian perineuriomatous) was apparent on immunohistochemistry, staining for epithelial membrane antigen, glut-1, claudin-1 being negative S-100 protein. Three members single family, history various malignant neoplasms. Included is lesion which neurofibromatous clearly visible hematoxylin- eosin-stained slides. unique that it manifested change protein-positive component, classified as peripheral tumor. component showed an epithelioid cell morphology.
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