Haybaeck Johannes, Roessner Albert
Institut für Pathologie, Univ.-Klinikum Magdeburg A.ö.R., Medizinische Fakultät, Otto-von-Guericke-Universität Magdeburg, Leipziger Straße 44, 39120, Magdeburg, Deutschland.
Institut für Pathologie, Neuropathologie und Molekuarpathologie, Medizinische Universität Innsbruck, Müllerstraße 44, 6020, Innsbruck, Österreich.
Pathologe. 2020 Mar;41(2):116-122. doi: 10.1007/s00292-020-00759-y.
Ewing sarcomas are highly malignant tumors that are mainly found in children and adolescents. In addition to early clinical diagnosis, correct histopathological and molecular genetic classification is the most important step. Although EWSR1-FLI1 fusion is by far the most common detectable change, there are also other representatives of the Ewing sarcoma family that cannot be distinguished histopathologically and immunohistochemically from classical Ewing sarcomas and that have different molecular genetic profiles. Although a precise molecular genetic differentiation of the various representatives of small round blue cell tumors does not yet lead to any change in the standard chemotherapy and surgical treatment applied, it does allow an estimation of the prognosis and will probably contribute in the future to an even more individualized treatment of Ewing sarcomas within the framework of personalized medicine.
尤因肉瘤是高度恶性肿瘤，主要见于儿童和青少年。除早期临床诊断外，正确的组织病理学和分子遗传学分类是最重要的步骤。尽管EWSR1-FLI1融合是目前最常见的可检测到的变化，但尤因肉瘤家族中还有其他成员，在组织病理学和免疫组织化学上无法与经典尤因肉瘤区分开来，且具有不同的分子遗传学特征。虽然对各种小圆蓝细胞肿瘤的不同代表进行精确的分子遗传学区分，目前尚未导致所应用的标准化疗和手术治疗发生任何改变，但它确实有助于预估预后，并且在未来个性化医疗的框架内，可能会为尤因肉瘤更个体化的治疗做出贡献。
