Woestenborghs Heidi, Debiec-Rychter Maria, Renard Marleen, Demaerel Philippe, Van Calenbergh Frank, Van Gool Stefaan, Sciot Raf
Department of Pathology, University Hospital, Catholic University of Leuven, Leuven, Belgium.
Int J Surg Pathol. 2005 Jan;13(1):93-7. doi: 10.1177/106689690501300114.
Peripheral primitive neuroectodermal tumor (PNET)/Ewing's sarcoma (ES) of the central nervous system is extremely rare and should be differentiated from central PNET and other small blue round cell tumors. We describe a case of a meningeal peripheral PNET/ES of the spinal cord in an 11-year-old boy. Immunohistochemically, the small blue round cell tumor showed expression of epithelial markers and of CD99, thus posing an important differential diagnostic problem with a poorly differentiated synovial sarcoma. Fluorescence in situ hybridization revealed rearrangement of the EWS gene, as seen in peripheral PNET/ES. Peripheral PNET/ES does occur in the central nervous system, but its diagnosis can be extremely difficult on morphologic and immunohistochemical grounds alone. Genetic analysis plays a key role in its distinction from other small blue round cell tumors.
中枢神经系统外周原始神经外胚层肿瘤（PNET）/尤因肉瘤（ES）极为罕见，应与中枢性PNET及其他小蓝圆细胞肿瘤相鉴别。我们报告一例11岁男孩脊髓的脑膜外周PNET/ES病例。免疫组化显示，该小蓝圆细胞肿瘤表达上皮标志物及CD99，因此与低分化滑膜肉瘤形成重要的鉴别诊断难题。荧光原位杂交显示EWS基因重排，这在外周PNET/ES中可见。外周PNET/ES确实可发生于中枢神经系统，但仅基于形态学和免疫组化进行诊断可能极为困难。基因分析在其与其他小蓝圆细胞肿瘤的鉴别中起关键作用。
