目的 探讨子宫内膜间质肉瘤(endometrial stromal sarcoma,ESS)的临床病理特征、诊断、鉴别诊断及预后.方法 对9例ESS患者进行临床、病理资料分析、免疫组化检测及随访.结果 患者年龄39～64岁,中位46.3岁.临床主要表现为阴道流血及子宫增大/占位.肿瘤直径2.3～11 cm,平均4.6 cm.光镜下8例呈低度恶性子宫内膜间质肉瘤(low grade endometrial stromal sarcoma,LGESS),均由类似增殖期子宫内膜间质肿瘤细胞构成,细胞密集,异型性不明显,呈不规则舌状或岛状浸润肌层,并伴较多薄壁螺旋小血管;1例为高度恶性子宫内膜间质肉瘤/未分化子宫内膜肉瘤(high grade endometrial stromal sarcoma/undifferentiated endometrial sarcoma,HGESS/UES),肿瘤细胞直接替代子宫肌层,具有明显的细胞异型性,无LGESS常见的螺旋小血管.免疫组化检测显示肿瘤细胞CD10、vimentin均阳性,PR、ER大部分阳性,SMA和desmin及h-Caldesmon为极少数局灶阳性,S-100、CD34均阴性.术后随访7例(平均53个月),只有1例HGESS/UES死亡.结论 ESS是女性生殖道很少见的一种恶性肿瘤,恶性度相差很大.确诊主要依靠其临床病理特点,并辅以免疫组化标记综合分析.诊断时要与子宫内膜间质结节、平滑肌肿瘤、低分化癌等鉴别.%Purpose To investigate the clinical characteristics, pathological features, diagnosis, differential diagnosis, and prognosis of endometrial stromal sarcoma ( ESS ). Methods Nine patients with ESS were analyzed on their clinical and pathological manifestations, key markers in immunohistochemistry, and follow-up. Results Patients aged from 39 to 64 years with a median age of 46. 3 years. Major clinical manifestations consisted of vaginal bleeding and enlarged uterus or occupied lesions in uterus. Diameters of these tumors varied from 2. 3 to 11 cm with the average of 4. 6 cm. Under light microscope, eight cases were diagnosed as low-grade of endometrial stromal sarcoma ( LGESS ), because these uterus were composed of endometrial stromal tumor cells which likely transformed from normal stromal cells in proliferation stage. Meanwhile, tumor cells densely populated without obvious atypia, invaded muscle layers with tongue-like, or irregular island-like shapes. There were also a lot of small and thin-walled spiral blood vessels in those tissues. One case was diagnosed as high-grade of endometrial stromal sarcoma or undifferentiated endometrial sarcoma ( HGESS/UES ), based on almost replacement of myometrium with tumor cells, and significant cellular atypia without small blood vessels which were common in LGESS. Immunohistochemical results showed that all tumor cells expressed both CD10 and vimentin, and most tumors expressed PR and ER. Meanwhile, SMA, desmin and h-caldesmon were only positive in a few tumor cells, but S-100, CD34 were negative. Seven patients were followed up after operation for an average of 53 months, only 1 case of HGESS/UES died. Conclusions ESS is a rare cancer involved in female genital tract. Malignant degree varies significantly among different patients. Accurate diagnosis mainly depends on the clinical features, histopathology, with help of a comprehensive analysis by immunohistochemistry. This disease should be discriminated from endometrial stromal nodules, smooth muscle tumors, and poorly differentiated carcinoma.
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