Katenkamp D
Institut für Pathologie, Friedrich-Schiller-Universität, Jena.
Praxis (Bern 1994). 1998 Aug 19;87(34):1043-9.
Malignant soft tissue tumors are classified and named according to cellular differentiation and thus the non-neoplastic soft tissue they imitate. The topical WHO classification already comprises more than 140 entities and tumor subtypes, but the process of defining new tumor variants will go on. Questions of nomenclature are discussed briefly with laying special emphasis on the non-undisputed concept of malignant fibrous histiocytomas. Without doubt, this diagnosis is made too frequently by which it has become to a collective name for unclassifiable pleomorphic sarcomas. For the time being nobody is able to say whether or not the malignant fibrous histiocytoma will remain an entity. Likely, fibrosarcomas and hemangiopericytomas are defined as exclusion diagnosis, as well. The diagnosis of malignant soft tissue tumors is based on recognizing the cellular line of differentiation. This is frequently possible at light microscopic level, sometimes additional diagnostic methods are required. The most important adjunct method is immunohistochemistry. Because aberrant differentiations and unexpected immunohistochemical reactions are known the use of a panel of antibodies is necessary. In the last years soft tissue tumors has increasingly been characterized by molecular biological methods. The results are of significance for understanding sarcoma pathogenesis and may be used for diagnosis, as well. Chromosome translocations are explained and rhabdomyosarcomas are taken as example for demonstrating the diagnostic significance of molecular biological/cytogenetic findings. Molecular biology may also aid in defining the histopathologic features of an entity as shown for intraabdominal desmoplastic small cell tumors. Eventually, heterogeneity in soft tissue sarcomas is addressed and discussed in view of its importance for diagnosis, classification and therapy as well as for development of sarcoma progression.
恶性软组织肿瘤根据细胞分化情况以及因此所模仿的非肿瘤性软组织进行分类和命名。世界卫生组织（WHO）的现行分类已包含140多种实体和肿瘤亚型，但定义新肿瘤变体的过程仍将继续。本文简要讨论了命名问题，特别强调了恶性纤维组织细胞瘤这一尚无定论的概念。毫无疑问，这种诊断过于频繁，以至于它已成为无法分类的多形性肉瘤的统称。目前，没有人能够确定恶性纤维组织细胞瘤是否会继续作为一个独立的实体存在。同样，纤维肉瘤和血管外皮细胞瘤可能也被定义为排除性诊断。恶性软组织肿瘤的诊断基于识别细胞分化谱系。这在光镜水平通常是可行的，有时需要额外的诊断方法。最重要的辅助方法是免疫组织化学。由于存在异常分化和意外的免疫组化反应，因此需要使用一组抗体。近年来，分子生物学方法越来越多地用于软组织肿瘤的特征描述。这些结果对于理解肉瘤的发病机制具有重要意义，也可用于诊断。本文解释了染色体易位，并以横纹肌肉瘤为例说明分子生物学/细胞遗传学结果的诊断意义。分子生物学还可以帮助确定一个实体的组织病理学特征，如腹内促结缔组织增生性小细胞瘤所示。最后，鉴于软组织肉瘤的异质性对诊断、分类和治疗以及肉瘤进展的重要性，本文对其进行了探讨。
