Vanhersecke Lucile, Linck Pierre-Antoine, Le Loarer François
Université de Bordeaux, 33400 Talence, France; Département de biopathologie, institut Bergonié, 33000 Bordeaux, France.
Département de biopathologie, institut Bergonié, 33000 Bordeaux, France.
Ann Pathol. 2022 Apr;42(3):227-241. doi: 10.1016/j.annpat.2022.01.017. Epub 2022 Feb 22.
Round cell sarcomas represent a diagnostic challenge for pathologists due to the poorly differentiated pattern of these high-grade tumors. Their diagnosis often requires large immunohistochemical panels and the use of molecular pathology. These tumors are largely dominated by Ewing sarcomas, but new families are now well characterized, including in decreasing frequency order in bone, BCOR-altered sarcomas, NFATc2-rearranged sarcomas, mesenchymal chondrosarcomas and more rarely CIC-rearranged sarcomas and myoepithelial tumors. This progress report presents microscopic, immunohistochemical and molecular features of these tumors previously named by the inappropriate term "Ewing-like" sarcomas, in order to enable any pathologist to perceive the morphological features of these sarcomas, to select the immunohistochemical panel that will lead to the diagnosis and to better guide the molecular approach needed to establish the final diagnosis.
圆形细胞肉瘤对病理学家来说是一个诊断挑战，因为这些高级别肿瘤的分化模式很差。它们的诊断通常需要大量免疫组织化学检测组合以及分子病理学的应用。这些肿瘤主要由尤因肉瘤主导，但现在新的肿瘤类型已得到充分表征，按在骨中出现频率递减顺序包括BCOR改变的肉瘤、NFATc2重排的肉瘤、间叶性软骨肉瘤，以及更罕见的CIC重排的肉瘤和肌上皮肿瘤。本进展报告介绍了这些以前被不恰当命名为“尤因样”肉瘤的肿瘤的微观、免疫组织化学和分子特征，以便任何病理学家都能识别这些肉瘤的形态特征，选择能得出诊断结果的免疫组织化学检测组合，并更好地指导建立最终诊断所需的分子检测方法。
