一例从神经纤维瘤到恶性周围神经鞘瘤的早期恶变病例

Poo Reum Kang, Y. Kim, Sun Ju Oh, H. Lee
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引用次数: 0

摘要

恶性周围神经鞘瘤(MPNST)是软组织肉瘤的一种,被认为起源于神经嵴细胞。I 型神经纤维瘤病(NF1)患者一生中发生恶性转化的风险约为 8-13%。NF1患者的神经纤维瘤可以通过手术切除,如果能够接近肿瘤,预后良好。我们曾接诊过一例 50 岁的 NF 女性患者,她的神经纤维瘤未完全切除,在首次手术后 3 个月迅速恶变为 MPNST。我们报告了这一病例,并简要回顾了相关文献。
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A Case of Early Malignant Change from Neurofibroma to Malignant Peripheral Nerve Sheath Tumor
Malignant peripheral nerve sheath tumor (MPNST) is one of the soft tissue sarcoma believed to originate from neural crest cells. The patients with neurofibromatosis type I (NF1) have about 8-13% of the lifetime risk of the malignant transformation. Neurofibroma on patients with NF1 can be surgically resected and has good prognosis if approach to the tumor is possible. We experienced a case of a 50-year-old woman with NF who had incompletely resected neurofibroma, which presented a rapid malignant transformation to MPNST, 3 months after the first surgery. We reported this case with a brief review of literature.
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