髓母肌母细胞瘤:一例成人病例报告及文献复习  

Medullomyoblastoma:report of an adult case and review of literatures

作　　者：柯昌庶[1] 邓仲端[1] 雷霆[2] 曾令成[2] 吴时敏[1] 万婕[1] 

机构地区：[1]华中科技大学同济医学院附属同济医院病理研究所病理科,武汉430030 [2]华中科技大学同济医学院附属同济医院病理研究所神经外科,武汉430030

出　　处：《中国现代神经疾病杂志》2008年第6期567-571,共5页Chinese Journal of Contemporary Neurology and Neurosurgery

摘　　要：目的报告1例临床少见的髓母肌母细胞瘤病例,探讨其临床病理学特征。方法对1例成人小脑髓母肌母细胞瘤患者的临床表现、组织形态学及免疫组织化学特征进行回顾分析,并复习相关文献。结果临床主要表现为持续性头痛,呈阵发性加重。MRI检查显示,病变位于右侧小脑半球及蚓部,呈混杂信号影,病灶边界欠清晰,有水肿改变,第四脑室明显受压,合并幕上脑室扩张、积水。手术切除肿瘤,组织形态学观察显示肿瘤细胞紧密排列,呈弥漫片状,密度较高,可见两种细胞成分,一种为卵圆形或短梭形小细胞,异型性明显,细胞核深染,几无细胞质;另一种为胞体较大的圆形或卵圆形肿瘤细胞,异型性明显,细胞核部分呈空泡状,有丰富的嗜伊红颗粒状细胞质。免疫组织化学染色显示,肿瘤细胞突触素、胶质纤维酸性蛋白、结蛋白、肌调节蛋白1、波形蛋白及神经元核抗原表达阳性,神经微丝和上皮膜抗原呈局灶性表达,而平滑肌肌动蛋白和白细胞共同抗原表达阴性;其中结蛋白和肌调节蛋白1表达阳性的肿瘤细胞,经证实为横纹肌分化。结论髓母肌母细胞瘤为髓母细胞瘤中极为少见的临床亚型,主要由髓母细胞瘤和横纹肌成分共同组成,好发于儿童,成人偶有发生,患者预后差。Objective To report a rare case with medullomyoblastoma and to explore the clinical pathological features of medullomyoblastoma. Methods Retrospectively analysed the clinical presentations, histomorphologieal and immunohistochemieal features of an adult case with eerebellar medullomyoblastoma. The related literatures were reviewed. Results A 49-year-old male patient presented with gradually exacerbated headache without nausea and vomiting for 4 months. Magnetic resonance imaging （MRI） showed that the lesion was located at the right lateral cerebellar hemisphere and vermis displaying heterogeneous signals. Its margin was not well demarcated and peri-lesionally edematous. The fourth ventricle of cerebrum was compressed and combined with supratentorial ventricular ectasia and dropsy. Grossly, greyish and whitish irregular brain tissue fragments were tender, measuring about 3 cm×3 cm × 3 cm in size. Histomorphologically, neoplastic cells were diffusely and compactly arranged, small tumor ceils＇ in ovoid and spindle shape, with hyperchromatic nuclei, fine granular chromatin and few cytoplasm were seen; the larger cells with plump eosinophilie granular cytoplasm and prominent nuclear atypia with vacuole were appreciated. Immunohistochemical staining showed that synapsin （Syn）, glial fibrillary acidic protein （GFAP）, desmin, myogenic differentiation protein 1 （MyoD1）, vimentin and neuronal nuclear antigen （Neu-N） were positive. Neurofilament （NF） and epithelial membrane antigen （EMA） were focally stained. However, the expression of smooth muscle aetin （SMA） and leucocyte common antigen （LCA） was negative. The expression of desmin and MyoDt was observed in the neoplastic ceils, demonstrating its rhabdomyogenic differentiation. Conclusion Medullomyoblastoma is a quite rare clinical subtype of medulloblastoma, featuring the co-existence of both medulloblastoma and rhabdomyogenic components in the tumor. It mainly involves the young children, but also occasionally occurs in adult popu

关 键 词：小脑肿瘤 髓母细胞瘤 横纹肌瘤 免疫组织化学 病理学 临床 

分 类 号：R739.4[医药卫生—肿瘤]