Mechtersheimer G, Renner M, Penzel R, Schirmacher P
Pathologisches Institut, Universitsklinikum Heidelberg, Im Neuenheimer Feld 220, 69120 Heidelberg, Deutschland.
Pathologe. 2011 Feb;32(1):47-56. doi: 10.1007/s00292-010-1400-4.
The conceptual evolution in the classification of pleomorphic high-grade sarcomas is a paradigm of how the integrative morphological, immunohistochemical and molecular genetic analysis has contributed to a clinical, prognostic and therapy-oriented characterization of this complex group of tumors. The clinical and prognostic relevance of a refined subtyping of pleomorphic high-grade sarcomas, which until recently was considered a mere academic exercise, is now undisputed. It is imperative to unequivocally differentiate sarcomas from non-sarcomatous, clearly defined malignancies to start adequate therapy. Furthermore, pleomorphic sarcomas which are particularly aggressive and prone to poor prognosis, have to be separated from sarcomas which, in contrast to the pleomorphic phenotype, are characterized by a less aggressive behavior. Also, morphologically pleomorphic but benign mesenchymal tumors must be recognized. Finally, it is important to promote the promising, array-based identification of diagnostic, prognostic and clinically relevant gene signatures on larger collections of pathomorphologically and clinically precisely defined subtypes of pleomorphic high-grade sarcomas.
多形性高级别肉瘤分类的概念演变是一个范例，展示了综合形态学、免疫组织化学和分子遗传学分析如何有助于对这一复杂肿瘤群体进行临床、预后和治疗导向的特征描述。多形性高级别肉瘤精细亚型分类的临床和预后相关性，直到最近还被认为只是一种学术行为，现在已无可争议。明确区分肉瘤与非肉瘤性、明确界定的恶性肿瘤对于开始适当治疗至关重要。此外，具有特别侵袭性且预后较差的多形性肉瘤必须与那些与多形性表型不同、行为侵袭性较小的肉瘤区分开来。同样，必须识别形态学上多形性但良性的间叶性肿瘤。最后，重要的是在更大规模的多形性高级别肉瘤病理形态学和临床精确定义亚型的样本集中，推动基于芯片的有前景的诊断、预后和临床相关基因特征识别。
