McCluggage W G, Date A, Bharucha H, Toner P G
Institute of Pathology, Royal Group of Hospitals Trust, Belfast, Northern Ireland.
Histopathology. 1996 Oct;29(4):369-74. doi: 10.1111/j.1365-2559.1996.tb01421.x.
An unusual uterine lesion is described in a patient with postmenopausal bleeding. Grossly, a yellow, polypoid mass projected into the uterine cavity. Histological examination showed a distinct biphasic pattern with areas of typical low-grade endometrial stromal sarcoma and areas where tumour cells were arranged in cords and trabeculae, resulting in a sex cord-like pattern. In these areas the cells assumed a rhabdoid morphology with eccentric vesicular nuclei, prominent nucleoli and eosinophilic hyaline cytoplasmic inclusions. Immunohistochemistry showed positive cytoplasmic staining of both components for vimentin, desmin and the cytokeratin marker CAM 5.2, but no staining for CEA and EMA. Electronmicroscopy revealed prominent paranuclear arrays of intermediate filaments. This is the second reported case of endometrial stromal sarcoma with rhabdoid differentiation and the first documented example of rhabdoid cells in sex cord-like areas. The report adds to the list of diverse neoplasms which may display a characteristic rhabdoid morphology and supports the hypothesis that extrarenal rhabdoid tumours are not a distinct clinicopathological entity. A diagnosis of malignant rhabdoid tumour of the uterus should be considered only when extensive sampling fails to disclose areas with an appearance typical of an endometrial stromal lesion.
本文描述了一名绝经后出血患者出现的一种不寻常的子宫病变。大体上，一个黄色的息肉样肿物突入子宫腔。组织学检查显示出一种独特的双相模式，既有典型的低级别子宫内膜间质肉瘤区域，也有肿瘤细胞呈条索状和小梁状排列的区域，形成性索样模式。在这些区域，细胞呈现出横纹肌样形态，核呈偏心泡状，核仁突出，胞质内有嗜酸性透明包涵体。免疫组化显示两种成分的波形蛋白、结蛋白和细胞角蛋白标记物CAM 5.2均呈胞质阳性染色，但癌胚抗原和上皮膜抗原无染色。电子显微镜显示核旁有明显的中间丝排列。这是第二例报道的具有横纹肌样分化的子宫内膜间质肉瘤病例，也是性索样区域出现横纹肌样细胞的首个记录实例。该报告增加了可能呈现特征性横纹肌样形态的多种肿瘤的名单，并支持肾外横纹肌样肿瘤不是一个独特的临床病理实体的假说。只有在广泛取材未能发现具有典型子宫内膜间质病变外观的区域时，才应考虑诊断为子宫恶性横纹肌样肿瘤。
