特殊形态的乳头状胶质神经元肿瘤

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目的报告一例中枢神经系统特殊形态的乳头状胶质神经元肿瘤患者的细胞学、组织病理学特点,以及超微结构和分子遗传学改变。方法回顾分析患者临床表现、影像学特点。印片、常规HE染色和电子显微镜观察肿瘤细胞形态、组织学特点和超微结构,荧光原位杂交试验柃测分子遗传学改变。结果临床主要表现为头痛、头晕伴恶心、呕吐,病程短。头部MRI检查显示右侧额颞叶腑实质内巨大囊实性占位性病变伴强化。组织病理学观察可见血管乳头中央和神经节细胞样细胞;肿瘤组织与脑白质分界较清楚,局部神经节细胞样细胞呈实片状排列,神经节细胞样细胞和嗜酸性颗粒小体呈散在分布,部分肿瘤细胞呈假乳头状排列,血管壁增厚伴透明变性;超微结构观察肿瘤细胞胞质丰富,内含较多粗面内质网和核糖体,核仁明显;免疫组织化学检测肿瘤细胞呈弥漫强阳性表达波形蛋门、CD34和巢蛋白,局灶表达AE1/AE3和上皮膜抗原,部分肿瘤细胞表达神经丝蛋白、CD56、突触素和胶质纤维酸性蛋白,个别细胞P53表达阳性,Ki-67抗原标记指数约为3%。荧光原位杂交试验可见散在多倍体细胞,但无表皮生长因子受体、表皮生长因子受休-1和人类表皮生长因子受体-2/拓扑异构酶Ⅱ的扩增。手术后局部放射治疗1个月,随访20个月未见复发征象。结论该例患者肿瘤细胞虽具备乳头状胶质神经元肿瘤的特点,但部分呈实片状分布,具有特殊的免疫组织化学表达,建议采用乳头状胶质神经元肿瘤中亚型——实性假乳头亚型命名。患者预后尚有待进一步随访观察。 Objective To report on the cytological, histopathological features of a patient with papillary glioma neurons in a special form of the central nervous system, as well as ultrastructural and molecular genetic changes. Methods Retrospective analysis of patients with clinical manifestations, imaging features. The morphology, histological features and ultrastructure of tumor cells were observed by HE staining and electron microscopy. Fluorescence in situ hybridization was used to detect the molecular genetic changes. Results The main clinical manifestations of headache, dizziness with nausea, vomiting, short course. MRI examination of the head showed massive cystic solid mass lesions in the right frontal temporal lamina propria with enhancement. Histopathological observation showed that the central of blood vessels and ganglion cell-like cells; tumor tissue and white matter more clearly, local ganglion cell-like cells were arranged in a plate-like arrangement, ganglion cells and eosinophilic granules were scattered , Part of the tumor cells were arranged in a pseudopapillary shape, with thickening of the vessel wall and degeneration of the transplanted cells. The ultrastructure of the tumor cells was characterized by abundant cytoplasm, abundant rough surface endoplasmic reticulum and ribosomes, obvious nucleoli, immunohistochemical detection of tumor The cells showed diffuse and strong positive expression of vimentin, CD34 and nestin, focal AE1 / AE3 and epithelial membrane antigen. Some tumor cells expressed neurofilament protein, CD56, synaptophysin and glial fibrillary acidic protein, and the expression of P53 in some cells Positive, Ki-67 antigen labeling index of about 3%. Fluorescence in situ hybridization showed scattered polyploid cells, but no epidermal growth factor receptor, epidermal growth factor Hugh-1 and human epidermal growth factor receptor-2 / topoisomerase II amplification. Local radiotherapy after surgery for 1 month, no signs of recurrence after 20 months of follow-up. Conclusion Although the tumor cells of this patient have the characteristics of papillary neuroglioma tumor, some of them are in a platelet distribution and have special immunohistochemical expression. It is suggested that the subtype-solidity of papillary neuroglial tumor False nipple subtype named. The prognosis of patients remains to be further follow-up observation.
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