Abstract：
Several defining molecular alterations have recently been identified underlying high-grade endometrial stromal sarcomas, such as YWHAE: NUTM2A/B fusions, ZC3H7B: BCOR fusions, and BCOR internal tandem duplication (ITD). BCOR is a useful immunohistochemical marker for identifying these tumors. A 37-year-old lady was presented with a 10-cm-sized tumor in the pouch of Douglas, involving the vaginal vault, bilateral adnexa, and peritoneum. A 53-year-old lady with a prior hysterectomy was presented with a 12-cm-sized tumor in the vault with abdominal deposits. Histopathological examination of both tumors revealed atypical cells comprising oval to spindle-shaped nuclei, a variable amount of myxoid stroma, and mitotic figures exceeding 10/10 high power fields. Immunohistochemically, the former tumor was diffusely positive for CD10, and the second tumor displayed patchy staining. Both tumors were positive for BCOR. Estrogen receptor (ER) showed variable staining in both tumors. By fluorescence in-situ hybridization (FISH), both tumors lacked YWHAE gene rearrangement. Both tumors had an aggressive clinical course, including extensive involvement This constitutes the first report of BCOR-positive high-grade sarcomas involving the female genital tract from our subcontinent. BCOR is a useful immunostain for identifying these relatively aggressive tumors. The differential diagnoses and the prognosis of these ultra-rare tumors are discussed herewith.
摘要：
最近已经确定了一些明确的分子改变，这些改变是高级别子宫内膜间质肉瘤的基础。如YWHAE：NUTM2A/B融合，ZC3H7B:BCOR融合,和BCOR内部串联复制(ITD)。BCOR是用于识别这些肿瘤的有用的免疫组织化学标记。一名37岁的女士在道格拉斯的袋子里出现了一个10厘米大小的肿瘤，涉及到阴道穹窿,双侧附件，还有腹膜.一名53岁的女士先前进行了子宫切除术，在穹窿中有一个12厘米大小的肿瘤，并有腹部沉积物。两种肿瘤的组织病理学检查均显示包含椭圆形至纺锤形核的非典型细胞，不同数量的黏液样基质,和有丝分裂数字超过10/10高倍场。免疫组织化学，前一个肿瘤CD10弥漫性阳性，第二个肿瘤呈斑片状染色。两种肿瘤均为BCOR阳性。雌激素受体（ER）在两种肿瘤中均显示出可变的染色。通过荧光原位杂交（FISH），两种肿瘤均缺乏YWHAE基因重排。两种肿瘤都有积极的临床过程，包括广泛参与这构成了涉及我们次大陆女性生殖道的BCOR阳性高级肉瘤的第一份报告。BCOR是用于鉴定这些相对侵袭性肿瘤的有用的免疫染色。本文讨论了这些超罕见肿瘤的鉴别诊断和预后。
