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目的探讨子宫内膜未分化及去分化癌的临床特点、病理特征、诊断及鉴别诊断。方法筛选2005—2015年间解放军总医院病理科可明确诊断为子宫未分化癌、去分化癌的病例,观察并总结此类病例的临床特点、病理形态特征、免疫组化染色结果及特殊染色在诊断中的作用,并进行相关文献复习。结果符合该诊断的病例共8例,其中去分化癌6例,未分化癌2例;中位年龄55岁,6例为绝经后发生。临床表现以不规则阴道流血为主。FIGO分期IC期3例,IIB期2例,Ⅲ期2例,Ⅳ期1例。镜下均见小圆细胞肿瘤成分伴地图样坏死,浸润子宫肌壁深层,1例可见较大的肿瘤细胞及瘤巨细胞。部分瘤细胞似浆细胞样或横纹肌样,黏附性差,核分裂象平均>20个/10HPF。6例可见脉管癌栓;6例经多点取材,于肿瘤周边见高~中分化内膜样癌成分(<10%),故诊断为去分化癌。免疫组化染色结果显示小圆细胞成分的部分区域vimentin(+),上皮性标记物pan-CK、EMA、CK7和CK8/18至少有一种局灶(+),部分肿瘤细胞CD99、CD56及Syn(+),7例CD138(+);但ER、PR均(-)。4例见微卫星不稳定现象。网织染色显示肿瘤细胞呈小巢状分布。3例FIGOⅢ~Ⅳ期患者均死于肿瘤,平均生存期13.3个月,其余被随访患者恢复良好。结论子宫未分化及去分化癌罕见,鉴别诊断时需多种上皮性标记物联合使用,且至少有一种上皮性标记物局灶阳性方可诊断。网织染色能较好地与间叶来源的肉瘤进行鉴别。其分子机制尚待进一步研究。
Objective To investigate the clinical features, pathological features, diagnosis and differential diagnosis of undifferentiated and dedifferentiated carcinoma of the endometrium. Methods To screen the cases of undifferentiated carcinoma and dedifferentiated carcinoma in the Department of Pathology, PLA General Hospital between 2005 and 2015. To observe and summarize the clinical features, pathological features, immunohistochemical staining and special staining of these cases In the role and review of relevant literature. Results In line with the diagnosis of a total of 8 cases, including dedifferentiated carcinoma in 6 cases, 2 cases of undifferentiated carcinoma; median age 55 years old, 6 cases of postmenopausal. The main clinical manifestations of irregular vaginal bleeding. 3 cases of FIGO stage IC, 2 cases of stage IIB, 2 cases of stage Ⅲ and 1 case of stage Ⅳ. Microscopically seen small round cell tumor components with the pattern of necrosis, infiltration of deep uterine muscle wall, 1 case showed larger tumor cells and tumor giant cells. Some tumor cells like plasmacytoid or rhabdomyosarcoma, poor adhesion, mitosis, the average number of> 20 / 10HPF. 6 cases of visible vascular thrombosis; 6 cases by multi-point drawing, see the tumor around the high-differentiated endometrial carcinoma (<10%), so the diagnosis of dedifferentiated cancer. Immunohistochemical staining showed that there were at least one focal (+) vimentin (+), epithelial markers pan-CK, EMA, CK7 and CK8 / 18 in part of the small cell components, CD99, CD56 and Syn (+), 7 cases of CD138 (+); but ER, PR (-). 4 cases see microsatellite instability. Mesh staining showed small nests of tumor cells. All 3 patients with FIGO Ⅲ ~ Ⅳ died of tumor, with an average survival of 13.3 months. The rest of the follow-up patients recovered well. Conclusion Undifferentiated and dedifferentiated uterine cancer is rare, the differential diagnosis of a variety of epithelial markers used in combination, and at least one epithelial marker focal positive for diagnosis. Mesh staining can better identify with sarcoma derived from mesenchyme. Its molecular mechanism needs further study.