Wardelmann Eva, Hartmann Wolfgang
Gerhard-Domagk-Institut für Pathologie, Universitätsklinikum Münster, Albert-Schweitzer-Campus 1, Gebäude D17, 48149, Münster, Deutschland.
Pathologe. 2021 May;42(3):281-293. doi: 10.1007/s00292-021-00935-8. Epub 2021 Apr 6.
The current WHO classification for tumors of soft tissue and bone includes numerous new entities, most often defined by novel molecular findings. In this article, we present translocation-positive tumors to broaden the spectrum of monomorphic mesenchymal neoplasias. The undifferentiated small round cell sarcomas are now assembled in their own separate chapter to underline their occurrence in both soft tissue and bone, emphasizing their morphologic, molecular, and biologic differences. Another interesting new group are tumors with GLI1 activation, which, however, have not yet been included into the WHO classification. NTRK-driven tumors present with a potential therapeutic target for several established inhibitors. Finally, there have been novel findings in rhabdomyosarcomas allowing more precise subtyping associated with different biological behavior.
世界卫生组织（WHO）目前的软组织和骨肿瘤分类包括众多新实体，大多由新的分子发现所定义。在本文中，我们展示易位阳性肿瘤以拓宽单形性间叶性肿瘤的谱。未分化小圆形细胞肉瘤现在被归在单独的一章中，以强调它们在软组织和骨中的发生，突出其形态学、分子和生物学差异。另一个有趣的新组是具有GLI1激活的肿瘤，然而，它们尚未被纳入WHO分类。NTRK驱动的肿瘤为几种已确立的抑制剂提供了潜在的治疗靶点。最后，横纹肌肉瘤有了新发现，使得与不同生物学行为相关的更精确亚型分类成为可能。
