Holzhausen H J, Stiller D, Sachs M
Institut für Pathologische Anatomie, Martin-Luther-Universität, Halle-Wittenberg, DDR.
Zentralbl Allg Pathol. 1988;134(4-5):489-97.
Described in this paper is a case of epithelioid haemangioendothelioma of soft tissue localised in the subcutis of the upper arm of a 47-year old man. The tumour was histologically characterized by cords and nests of plump epithelioid cells separated from each other by typical myxohyaline stroma. Light microscopy revealed also definite vasoformative areas and solid regions with vacuolization which were indicative of so-called intracytoplasmic vacuoles. Atypical nuclei were conspicuous findings. Ultrastructural and immunohistochemical investigations demonstrated the endothelial origin of the lesion. Immunohistochemical investigations also revealed the presence of cellular areas which produced positive response to "factor-VIII-associated antigen". Ultrastructurally the tumour cells exhibited densely packed microfilaments, 8 nm to 10 nm in thickness, and intracytoplasmic vacuoles. The latter were lined and delimited by single membranes, and some of these exhibiting luminal microvilli. The tumour cells exhibited typical pinocytotic vacuoles, with the cords or nests being encircled by thin basal membranes. Typical Weibel-Palade bodies were detectable in some cells. Also recordable from certain cells were intracytoplasmic crystalloid inclusion bodies of a kind never described before in the context of tumour cells of epithelioid haemangioendothelioma. Those inclusions were composed of substructural units of parallel electrodense lines, 19nm to 21nm in periodicity. The nature of epithelioid haemangioendothelioma and its relations with intravascular bronchiolo-alveolar tumours (IVBAT) and myxoid angioblastomatosis of bone were discussed in some detail.
本文描述了一例软组织上皮样血管内皮瘤,位于一名47岁男性上臂皮下。肿瘤组织学特征为丰满的上皮样细胞条索和巢团,由典型的黏液透明基质分隔。光镜检查还发现明确的血管形成区域和有空泡化的实性区域,提示所谓的胞质内空泡。非典型核是明显的表现。超微结构和免疫组化研究证实了病变的内皮起源。免疫组化研究还显示存在对“因子VIII相关抗原”呈阳性反应的细胞区域。超微结构上,肿瘤细胞显示出密集排列的微丝,厚度为8纳米至10纳米,还有胞质内空泡。后者由单层膜衬里和界定,其中一些有腔内微绒毛。肿瘤细胞显示典型的吞饮空泡,条索或巢团被薄的基底膜环绕。在一些细胞中可检测到典型的Weibel-Palade小体。在某些细胞中还可记录到上皮样血管内皮瘤肿瘤细胞中从未描述过的一种胞质内晶体包涵体。这些包涵体由平行的电子致密线亚结构单位组成,周期为19纳米至21纳米。本文还详细讨论了上皮样血管内皮瘤的性质及其与血管内细支气管肺泡肿瘤(IVBAT)和骨黏液样血管母细胞瘤的关系。