皮肤神经纤维瘤病恶变1例

A case of cutaneous neurofibromatosis with malignant change

报告1例少见的皮肤神经纤维瘤病恶变。患者为一71岁老年男性。主要表现为全身性皮肤结节，最大直径达7cm；,镜检：大多表现为典型的神经纤维瘤，但部分肿瘤显示出明显的恶性特征：瘤细胞异形显著，病理性核分裂明显。免疫组化采用S－100、Vim、NSE等抗体证明其来源于神经组织。诊断为皮肤神经纤维瘤病伴恶性神经鞘瘤。

A rare case with cutaneous neurofibromatosis (NF) with malignant change was reported. The patiant was a 71-year-old man. The chief clinical sign was the cutaneous nodules throughout the whole bady. The largest nodule reached 7 cm in diameter. His-tologically, besides typical neurofibroma appearances. there were prominant malignantfeatures in some tumors. striking atypia of tumor cells and obvious pathologic mitosisfeatures. Immunohistochemial study with S- 100. NSE. Vim et al. antibodies had iden-tified that these malignant cells were derived from nervous tissue. The diagnosis wascutaneous neurofibromatosis with malignant neurilemoma.