促纤维化小圆细胞肿瘤5例临床病理、免疫组化及电镜观察  

Desmoplastic small round cell tumor:A report of 5 cases

作　　者：高冬霞[1] 廖松林[1] 石雪君[2] 回允中[3] 

机构地区：[1]北京医科大学病理学系,100083 [2]北京医科大学第一附属医院病理科,100034 [3]北京医科大学第二附属医院病理科,100044

出　　处：《临床与实验病理学杂志》2000年第5期353-356,共4页Chinese Journal of Clinical and Experimental Pathology

摘　　要：目的 :观察 5例促纤维化小圆细胞肿瘤的临床病理特点 ,提高对此类肿瘤的认识。方法 :常规组织学 (HE)、免疫组化(ABC法 )和电镜技术检测结合临床资料综合分析。结果 :5例中 2例多次局部复发 ,2例在随访中 ,1例确诊后 4个月死亡。该肿瘤组织学特点为肿瘤组织呈单结节或多结节状浸润生长 ,伴有明显致密的成熟纤维性间质增生 ,肿瘤细胞主要为大小比较一致的小圆形细胞 ,呈团状或巢状埋于增生纤维间质中 ,少数病例部分区域肿瘤细胞显示有不同分化特点。免疫组化 :5例均有上皮表达 (AE1/AE3或EMA) ,3例表达NSE ,1例间质表达desmin ,1例间质表达vimentin。其中 1例做了电镜检查 ,细胞内可见张力原纤维和桥粒。结论 :(1)促纤维化小圆细胞肿瘤发病年龄跨度较大 ,发生部位可在腹腔也可在腹腔外 ;(2 )该肿瘤具有独特的组织学特点 ,大部分肿瘤细胞为无明确特点分化的上皮性小细胞 ,并埋于显著纤维化的间质中 ;(3)少数病例肿瘤细胞可因发生部位不同可有不同的特殊分化 ,较成熟特殊分化是预后好的标志 ;(4 )此瘤预后与发生部位、分裂活性及分化有关。Purpose To recognize the characteristics of desmoplastic small round cell tumor (DSRCT). Methods Five cases of DSRCT were studied using usual histologic (HE staining), immunohistochemical (ABC method), electron microscopic techniques and clinical data. Results Two cases recurred many times at the same site. Among the two who were followed, one died four months after diagnosis. This tumor occurred not only in the abdomen but also at other sites. Histologically, the tumor consisted of nests of small round cells with scant cytoplasm embedded in a densely fibrotic stroma. At different sites there were various differentiations in a part of tumor. Immunohistochemically, all of the 5 cases were positive for AE1/AE3 or EMA, 3 cases expressed NSE, stroma of 2 cases expressed desmin and vimentin respectively. One case was observed by electron microscope and tonofilaments in cells and desmosomes between cells were seen. Conclusions (1)Patients with DSRCT range widely in age. This tumor occurs both in the abdomen and at other sites. (2) Microscopically, the tumors typically have a characteristic appearance of nests of small cells embedded in a desmoplastic stroma. (3) A few tumor cells show various differentiation at diferent sites. (4) The prognosis of the patients is connected with the tumors site, mitotic activity and differentiation.

关 键 词：促纤维化小圆细胞肿瘤 免疫组织化学 电子显微镜 

分 类 号：R730.26[医药卫生—肿瘤]