The 2020 WHO Classification of Soft Tissue Tumours: news and perspectives.
The 2020 WHO Classification of Soft Tissue Tumours: news and perspectives.
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DOI：
10.32074/1591-951x-213
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发表时间：
2021-04
期刊：
影响因子：
3.5
通讯作者：
Dei Tos AP
中科院分区：
文献类型：
--
作者：
Sbaraglia M;Bellan E;Dei Tos AP
间叶性肿瘤是诊断病理学中最具挑战性的领域之一，分类方案的改进在提高病理诊断的质量和治疗方案方面起着关键作用。最近出版的世界卫生组织软组织肿瘤和骨的新分类是朝着改进诊断标准化迈出的重要一步。重要的是，2020年世卫组织分类已向专业临床医生开放，这进一步有助于强调病理诊断作为适当治疗的理论基础的关键价值。已经介绍了几个相关的进展。为了改善对孤立性纤维肿瘤临床行为的预测，已经实施了风险评估方案。考虑到NTRK抑制方面的最新治疗进展，NTRK重排的软组织肿瘤现在被列为“新兴实体”。这一决定引发了关于“肿瘤实体”的定义以及精确肿瘤学的“病理学不可知论”方法的后果的激烈辩论。考虑到它们不同的临床病理特征，未分化的圆细胞肉瘤现在与尤文肉瘤分开，并根据潜在的基因重排分为三个主要亚组(CIC、BCLR和非ETS融合肉瘤)。重要的是，为了避免潜在的混淆，胃肠道间质瘤等肿瘤实体在不同的WHO分组中被统一处理。病理诊断代表了形态、免疫组织化学和分子特征的综合，是临床决策的关键因素。世卫组织的分类是促进多学科的关键工具，激励病理学家、遗传学家和临床医生共同努力，将新的病理发现转化为更有效的治疗方法。
Mesenchymal tumours represent one of the most challenging field of diagnostic pathology and refinement of classification schemes plays a key role in improving the quality of pathologic diagnosis and, as a consequence, of therapeutic options. The recent publication of the new WHO classification of Soft Tissue Tumours and Bone represents a major step toward improved standardization of diagnosis. Importantly, the 2020 WHO classification has been opened to expert clinicians that have further contributed to underline the key value of pathologic diagnosis as a rationale for proper treatment. Several relevant advances have been introduced. In the attempt to improve the prediction of clinical behaviour of solitary fibrous tumour, a risk assessment scheme has been implemented. NTRK-rearranged soft tissue tumours are now listed as an “emerging entity” also in consideration of the recent therapeutic developments in terms of NTRK inhibition. This decision has been source of a passionate debate regarding the definition of “tumour entity” as well as the consequences of a “pathology agnostic” approach to precision oncology. In consideration of their distinct clinicopathologic features, undifferentiated round cell sarcomas are now kept separate from Ewing sarcoma and subclassified, according to the underlying gene rearrangements, into three main subgroups (CIC, BCLR and not ETS fused sarcomas) Importantly, In order to avoid potential confusion, tumour entities such as gastrointestinal stroma tumours are addressed homogenously across the different WHO fascicles. Pathologic diagnosis represents the integration of morphologic, immunohistochemical and molecular characteristics and is a key element of clinical decision making. The WHO classification is as a key instrument to promote multidisciplinarity, stimulating pathologists, geneticists and clinicians to join efforts aimed to translate novel pathologic findings into more effective treatments.