Malignant progression of an extraventricular neurocytoma arising from
Malignant progression of an extraventricular neurocytoma arising from
复制标题
室外神经细胞瘤的恶性进展
DOI：
10.1111/neup.12533
复制
发表时间：
2019
期刊：
影响因子：
2.3
通讯作者：
Morioka M
中科院分区：
文献类型：
--
作者：
Sugita Y;Furuta T;Komaki S;Ohshima K;Sakata K;Morioka M
我们报告一位34岁女性，发生于第viii脑神经的罕见脑室外神经细胞瘤（EVN）。患者有20年的听力损失和面瘫病史。计算机断层扫描显示左侧桥小脑角（CPA）有一个3 cm的强化病灶。手术时发现肿瘤起源于耳蜗和前庭神经。肿瘤被部分切除。组织学上，肿瘤由均匀的细胞组成，细胞核卵圆形，胞质稀少。免疫组化结果显示，肿瘤细胞突触素阳性，但胶质纤维酸蛋白和S - 100蛋白阴性。Ki‐67标记指数为0%。术后12年MRI显示左侧CPA肿瘤复发。肿瘤被部分切除，并给予放射治疗。组织学上，肿瘤由圆形细胞组成，轻度异型性，1个有丝分裂/20个高倍视场（HPF）。免疫组织化学结果显示，除了Ki‐67标记指数（3%）外，肿瘤细胞显示与第一次手术样本相同的结果。第二次手术12年后，MRI显示左侧CPA和脑干周围肿瘤再次复发。肿瘤被部分切除。组织学上，肿瘤由间变性短梭形细胞和5个有丝分裂/10 HPF组成。免疫组化结果与早期手术样本基本相同。然而，Ki‐67标记指数为20%。此外，与早期的样本相比，第三个样本的肿瘤细胞对GAB1（生长因子受体结合蛋白2相关结合蛋白1）的表达更强烈、更广泛。电镜下可见大量细胞突，核致密，囊泡和微管清晰。GAB1免疫染色也提示恶性进展可能与sonic hedgehog信号通路有关。据我们所知，这是第一例由第8脑神经引起的恶性进展的EVN。
A rare case of extraventricular neurocytoma (EVN) arising from the VIIIth cranial nerve in a 34‐year‐old woman is reported. The patient had a 20‐year history of hearing loss and facial palsy. Computed tomography showed a 3‐cm enhancing lesion in the left cerebellopontine angle (CPA). At operation, the tumor was seen to originate from the cochlear and vestibular nerves. The tumor was subtotally resected. Histologically, the tumor consisted of uniform cells with oval to round nuclei and scant cytoplasm. Immunohistochemically, the tumor cells were positive for synaptophysin, but negative for glial fibrillary acid protein and S‐100 protein. The Ki‐67 labeling index was 0%. Twelve years after the operation, magnetic resonance imaging (MRI) showed tumor recurrence at the left CPA. The tumor was subtotally resected, and radiation therapy was given. Histologically, the tumor consisted of round cells with mild atypia and one mitosis/20 high‐power fields (HPF). Immunohistochemically, tumor cells showed the same findings as the first operation sample, except for the Ki‐67 labeling index (3%). Twelve years after the second operation, MRI showed a second tumor recurrence at the left CPA and surroundings of the brain stem. The tumor was subtotally resected. Histologically, the tumor consisted of anaplastic short spindle cells and five mitoses/10 HPF. The immunohistochemical findings were almost the same as the earlier operation samples. However, the Ki‐67 labeling index was 20%. In addition, tumor cells from the third specimen were more strongly and more diffusely positive for GAB1 (growth factor receptor‐bound protein 2‐associated binding protein 1) compared to those of the earlier specimens. Electron microscopy showed the presence of numerous cell processes with a dense core and clear vesicles and microtubules. GAB1 immunostaining also indicated that malignant progression might be associated with the sonic hedgehog signaling pathways. To the best of our knowledge, this is the first report of an EVN arising from the VIIIth cranial nerve with malignant progression.