Kazan Saim, Göksu Ethem, Mihci Ercan, Gökhan Güzide, Keser Ibrahim, Gürer Inanc
Department of Neurosurgery, Akdeniz University, Antalya, Turkey.
J Neurosurg. 2007 Apr;106(4 Suppl):308-11. doi: 10.3171/ped.2007.106.4.308.
An atypical teratoid/rhabdoid tumor of the central nervous system (CNS) is a rare, aggressive neoplasm found in infants and children that has similar characteristics to CNS primitive neuroectodermal tumors/medulloblastomas. The authors present the case of a patient with an atypical teratoid/rhabdoid tumor and discuss the imaging, histopathological, immunohistochemical, and cytogenetic findings. Tumor cells displayed positive reactions for vimentin, epithelial membrane antigen, and cytokeratin, and they displayed no reaction for glial fibrillary acidic protein, desmin, and actin. The karyotype was 46, XY. The phenotype of an atypical teratoid/rhabdoid tumor appears heterogeneous when examined by histological, immunohistochemical, and genetic analysis. The authors describe the case of a 4-year-old boy who harbored an atypical teratoid/rhabdoid tumor in the clivus, which appeared as a chordoma on neuroimages. To their knowledge, this location of an atypical teratoid/rhabdoid tumor has not been described in the literature.
中枢神经系统非典型畸胎样/横纹肌样肿瘤（CNS）是一种罕见的侵袭性肿瘤，见于婴幼儿，其特征与中枢神经系统原始神经外胚层肿瘤/髓母细胞瘤相似。作者报告了一例非典型畸胎样/横纹肌样肿瘤患者的病例，并讨论了影像学、组织病理学、免疫组化和细胞遗传学检查结果。肿瘤细胞波形蛋白、上皮膜抗原和细胞角蛋白呈阳性反应，而胶质纤维酸性蛋白、结蛋白和肌动蛋白呈阴性反应。核型为46，XY。通过组织学、免疫组化和基因分析检查时，非典型畸胎样/横纹肌样肿瘤的表型显得异质性。作者描述了一名4岁男孩的病例，该男孩斜坡部患有非典型畸胎样/横纹肌样肿瘤，神经影像上表现为脊索瘤。据他们所知，文献中尚未描述非典型畸胎样/横纹肌样肿瘤的这一部位。
