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成人髓母细胞瘤伴肌原性分化
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成人髓母细胞瘤伴肌原性分化
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Journal Article
成人髓母细胞瘤伴肌原性分化
2015
Overview
目的报告1例成人髓母细胞瘤伴肌原性分化病例,探讨其临床病理学特征,并复习文献,以提高对此类肿瘤的诊断与鉴别诊断能力。方法与结果女性患者,32岁,临床表现为反复口角歪斜、面部麻木6年余。T1WI显示小脑蚓部和脑干背侧混杂信号影,并突向第四脑室;增强扫描显示病灶内类圆形强化结节。手术全切除肿瘤。术中见肿瘤位于小脑蚓部,突向第四脑室并侵及脑干。组织学形态,卵圆形核瘤细胞呈片状密集或散在分布,可见＂菊形团＂样结构,胞质丰富、嗜酸性,胞核偏位、异型性明显,可见核仁或染色质深染,核分裂象易见,伴出血。免疫组织化学染色,肿瘤细胞弥漫性表达整合酶相互作用分子1、突触素、嗜铬素A、人互联蛋白神经元中间丝蛋白α、神经微丝蛋白、巢蛋白、β-联蛋白和P53,部分表达结蛋白、神经元核抗原和S-100蛋白,不表达胶质纤维酸性蛋白、少突胶质细胞转录因子2、CD99、广谱细胞角蛋白、上皮膜抗原、肌调蛋白1、肌浆蛋白、肌特异性肌动蛋白和平滑肌肌动蛋白,Ki-67抗原标记指数约为10%。病理诊断：髓母细胞瘤伴肌原性分化（WHOⅣ级）。术后未接受放射治疗或药物化疗,随访9个月未见肿瘤复发。结论髓母细胞瘤是一种常见于儿童小脑的恶性侵袭性胚胎性肿瘤。发生于成人的髓母细胞瘤伴肌原性分化病例少见,含原始神经外胚层细胞和横纹肌母细胞成分是其特点,诊断时应注意与中枢神经系统原始神经外胚层肿瘤、非典型畸胎样/横纹肌样肿瘤和横纹肌肉瘤相鉴别。
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