Stiller D, Holzhausen H J
Institut für Pathologische Anatome, Martin-Luther-Universität Halle - Wittenberg, DDR.
Zentralbl Allg Pathol. 1988;134(4-5):449-66.
Rhabdomyosarcomas may be easily diagnosed by light microscopy when tumor cells exhibit cross striations typical of skeletal muscle cells. But in many cases the histological diagnosis is difficult because the tumors are predominantly composed of undifferentiated elements and only single cells can be seen which show an eosinophilic cytoplasm suggesting different steps of a possible rhabdomyoblastic differentiation. Using the electron microscope seven embryonal rhabdomyosarcomas, one alveolar rhabdomyosarcoma including one lymph node metastasis and one pleomorphic rhabdomyosarcoma were analysed in order to study the submicroscopical organization of diagnostic cellular features, resp. the distribution and arrangement of cytoplasmic filaments. The following lines of cellular differentiation could be distinguished: 1. Development of differentiated myoblasts and satellite cells: -- primitive, undifferentiated tumor cells: small round cells with scanty cytoplasm containing few thin filaments (4 to 6 nm) and intermediate-type filaments (10 nm), -- round or slightly spindle-shaped myoblast-like tumor cells: cells with moderate cytoplasm exhibiting irregularly arranged thin and intermediate-type filaments and only some thick filaments (15 nm), -- myotube-like cells: long extended cells (strap shaped cells) revealing thin and thick filaments, Z-line material and different stages of myofibrillar organization, -- well-differentiated myoblasts: long extended cells showing typical cross-striations which correspond to well-formed sarcomeres with I-bands (Z-lines with extending thin filaments) and A-bands, which are subdivided into H- and M-bands, -- satellite cells of typical ultrastructure associated with differentiated myoblasts by a common basement membrane present in one case. 2. Development of aberrant myoblasts and giant cells: -- round myoblasts: cells with increased cytoplasm containing thin and thick filaments, primitive Z-lines, which were not organized into sarcomeres as well as unaligned sarcomeres, -- large round myoblasts and giant cells: cells with abundant cytoplasm containing irregularly distributed thin and thick filaments, primitive Z-lines and haphazardly arranged sarcomeres, which did not appear as cross-striations by light microscopy. The ultrastructure of tumor cells is discussed with regard to the degree of differentiation and their light microscopic appearance. The scale of cellular features in rhabdomyosarcomas could be also correlated with normal fetal myogenesis. Furthermore this confirms the important role of electron microscopy in the differential diagnosis of other small, primitive and dark-cell tumors.
当肿瘤细胞呈现出骨骼肌细胞典型的横纹时,横纹肌肉瘤通过光学显微镜很容易诊断。但在许多情况下,组织学诊断很困难,因为肿瘤主要由未分化成分组成,只能看到单个细胞,其嗜酸性细胞质提示可能的横纹肌母细胞分化的不同阶段。为了研究诊断性细胞特征的亚微观组织,即细胞质细丝的分布和排列,使用电子显微镜对7例胚胎性横纹肌肉瘤、1例肺泡性横纹肌肉瘤(包括1例淋巴结转移)和1例多形性横纹肌肉瘤进行了分析。可以区分以下细胞分化途径:1. 分化的成肌细胞和卫星细胞的发育:——原始未分化肿瘤细胞:小圆形细胞,细胞质稀少,含有少量细肌丝(4至6纳米)和中间型肌丝(10纳米);——圆形或略呈梭形的成肌细胞样肿瘤细胞:细胞质适中的细胞,表现出不规则排列的细肌丝和中间型肌丝,只有一些粗肌丝(15纳米);——肌管样细胞:长梭形细胞(带状细胞),有细肌丝和粗肌丝、Z线物质以及肌原纤维组织的不同阶段;——分化良好的成肌细胞:长梭形细胞,显示典型横纹,对应于具有I带(带有延伸细肌丝的Z线)和A带的成熟肌节,A带又分为H带和M带,在1例中可见通过共同基底膜与分化的成肌细胞相关的典型超微结构的卫星细胞。2. 异常成肌细胞和巨细胞的发育:——圆形成肌细胞:细胞质增加的细胞,含有细肌丝和粗肌丝、原始Z线,未组织成肌节以及排列不齐的肌节;——大圆形成肌细胞和巨细胞:细胞质丰富的细胞,含有不规则分布的细肌丝和粗肌丝、原始Z线和杂乱排列的肌节,在光学显微镜下未表现为横纹。根据分化程度及其光学显微镜外观对肿瘤细胞的超微结构进行了讨论。横纹肌肉瘤中细胞特征的范围也可以与正常胎儿肌发生相关。此外,这证实了电子显微镜在其他小的、原始的和暗细胞肿瘤的鉴别诊断中的重要作用。