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目的探讨非典型脑室外神经细胞瘤的临床病理特征。方法对1例非典型脑室外神经细胞瘤的临床表现、影像学、组织形态、免疫组化等进行分析并复习相关文献。结果患者男性,32岁。间断癫发作2年余。术前MRI提示右侧颞叶巨大囊实性占位。组织学上,瘤细胞为弥漫一致的中等大小透明细胞,其间可见小片状分布的神经毡样结构,细胞有一定异型性,核分裂象5个/10HPF,可见血管增生及栅栏样坏死。免疫组化显示瘤细胞Syn和S-100弥漫(+),少数瘤细胞GFAP、nestin和Neu-N(+),而Oligo-2、EMA、neurofilament和p53均(-),Ki-67约为20%。结论非典型脑室外神经细胞瘤临床上罕见,组织学形态上类似间变性少突细胞瘤、间变性室管膜瘤及多形性胶质母细胞瘤等,易误诊。特征性的组织学特点及免疫表型有助于鉴别。
Objective To investigate the clinicopathological features of atypical extracerebral neuroblastoma. Methods The clinical manifestations, imaging, histomorphology and immunohistochemistry of atypical extracerebral neuroblastoma were analyzed and the related literatures were reviewed. Results The patient was male, 32 years old. Intermittent epileptic seizures more than 2 years. Preoperative MRI prompts the right temporal lobe huge cystic solid occupancy. Histologically, the tumor cells were diffusely uniform medium sized transparent cells. During the process, there was a small patchy neurofibrillar structure. The cells had a certain atypia. The mitotic figures were 5/10 HPF, showing vascular proliferation and palisade-like necrosis. Immunohistochemistry showed that the tumor cells Syn and S-100 diffuse (+), a few tumor cells GFAP, nestin and Neu-N (+), while Oligo-2, EMA, neurofilament and p53 20%. Conclusions Atypical extracerebral neuroblastoma is rare in clinical practice. It is easy to be misdiagnosed as histological morphological resemblance to interstitial cystic degeneration, anaplastic ependymoma and glioblastoma multiforme. The characteristic histological features and immunophenotype contribute to the identification.