Study of Malignant Peripheral Nerve Sheath Tumor in Cerebellopontine Angle
Study of Malignant Peripheral Nerve Sheath Tumor in Cerebellopontine Angle
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DOI：
10.1097/scs.0000000000000622
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发表时间：
2014-03-01
影响因子：
0.9
通讯作者：
Feng, ChunGuo
中科院分区：
文献类型：
--
作者：
Hong, WenMing;Cheng, HongWei;Feng, ChunGuo
恶性周围神经鞘瘤(MPNST)是一种非常罕见的软组织肉瘤，通常发生于躯体软组织或周围神经。桥小脑角的原发MPNST非常罕见，到目前为止只有一例报道。在此，我们报告一例罕见的桥小脑角MPNST病例，患者为25岁男性，表现为头晕、左侧面部麻木和耳鸣。入院后，肿瘤接受完全手术切除，然后辅以化疗和放疗。组织学表现为恶性梭形细胞，免疫组织化学S-100局灶性阳性，诊断为MPNST。据报道，此病例罕见且存在于桥小脑，说明了诊断和治疗MPNST的困难，据我们所知，这种疾病在软组织肉瘤中从未被描述过。
Malignant peripheral nerve sheath tumors (MPNSTs) are very rare soft tissue sarcomas, usually arising from somatic soft tissues or peripheral nerves. Primary MPNST of the cerebellopontine angle is extremely rare, with only a single case reported so far. Here, we report an unusual case of MPNST in cerebellopontine angle in a 25-year-old man presented with dizziness, left facial numbness, and tinnitus. After hospitalization, the tumor was treated with complete surgical excision followed by adjuvant chemotherapy and radiotherapy. Histologically, the tumor showed malignant spindle cells, which were with focal S-100 positivity on immunohistochemistry, and a diagnosis of the MPNST was made. This case is being reported for its rarity and presence in cerebellopontine and illustrated the difficulties in the diagnosis and treatment of MPNST, which to the best of our knowledge, has not been described before in the soft tissue sarcomas.