目的探讨伴髓上皮瘤和间叶分化大脑内含有神经毡和菊形团的胚胎性肿瘤的临床病理特点、诊断与鉴别诊断。方法对1例发生在左侧颞顶叶的含有神经毡和菊形团的胚胎性肿瘤伴髓上皮瘤和间叶分化进行组织学、免疫组化观察,并结合文献复习。结果患儿男性,5岁。头颅CT示左侧颞顶叶肿瘤性病变伴钙化,左侧颞骨骨质破坏,考虑左颞顶叶占位。光镜下瘤组织由疏密相间区域构成,致密区域见原始小圆核或短梭形瘤细胞呈片状密集分布,核染色质深,异型性明显,见核分裂象伴多灶性坏死,可见横纹肌样细胞,部分区域见柱状上皮构成的原始神经管样结构或Homer-Wright菊形团形成伴结缔组织增生;稀疏区见分化良好的胶质神经毡样基质内有小型神经元样瘤细胞或节细胞样瘤细胞散在分布。免疫组化:原始神经管样结构基底部vimentin(+),原始瘤细胞和横纹肌样细胞nestin、INI-1和p53(+),横纹肌样细胞NF、Syn、MAP2和Cg A(+),Ki-67为局灶20%,原始神经管样结构CK和EMA(-),横纹肌样细胞SMA(-),横纹肌样细胞GFAP和NSE灶(+),小型神经元样瘤细胞S-100和Neu N(+),Olig2(-/+),β-catenin和desmin(-)。结论大脑内含有多量神经毡和真性菊形团的胚胎性肿瘤伴髓上皮瘤和间叶分化确诊依赖于影像、组织学和免疫组化,需要与髓母细胞瘤、非典型畸胎样/横纹肌样瘤、室管膜母细胞瘤和脉络丛乳头状癌鉴别。 Objective To investigate the clinicopathological features, diagnosis and differential diagnosis of embryonal neoplasms with neurofilament and chrysidome in the accompanying myeloma and mesenchymal brain. Methods Histological and immunohistochemical studies of one case of embryonal tumor with neurofilament and chrysomme in the left temporal parietal region with myeloma and mesenchymal differentiation were performed and reviewed. Results children male, 5 years old. Head CT showed left temporal parietal tumor lesions with calcification, left temporal bone destruction, consider the left temporal roof of the place. Under light microscopy, the tumor tissue consisted of dense and dense interphase area. In the dense area, the primitive orbital nuclear or short shuttle tumor cells were distributed in the form of flake. The chromatin of the nucleus was deep and the atypia was obvious. See mitosis with multifocal necrosis Rhabdomyosarcoma cells, primitive neural tube-like structures composed of columnar epithelium or Homer-Wright damascena formed connective tissue hyperplasia in some areas. In well-differentiated glial nerve-like matrix, there are small neuron-like tumor cells or Cell-like tumor cells scattered scattered. Immunohistochemistry: Vimentin (+), nestin, INI-1 and p53 (+) and rhabdomyostrophic cells NF, Syn, MAP2 and Cg A -67 was localized in 20% of the lesions. The primary neural tube-like structures CK and EMA (-), striated muscle cells SMA (-), striated muscle cells GFAP and NSE foci (+), small neuronal tumor cells S-100 and Neu N (+), Olig2 (- / +), β-catenin and desmin (-). Conclusions The diagnosis of myeloma and mesenchymal tumor with a large number of neurofilament and true chrysanthemum in the brain depends on the imaging, histology and immunohistochemistry. The diagnosis of medulloblastoma, atypical teratoid / rhabdomyosarcoma Like tumors, ependymoma and choroid plexus papillary carcinoma differential identification.