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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。
Objective : To observe the clinicopathological features of 5 cases of pro-fibrotic small round cell tumors and improve the understanding of such tumors. METHODS: General histology (HE), immunohistochemical (ABC) and electron microscopy techniques were combined with comprehensive analysis of clinical data. Results: Two of 5 cases had local recurrence, 2 cases were followed up, and 1 died 4 months after diagnosis. The histological characteristics of the tumor is that the tumor tissue shows single-nodular or multinodular infiltrative growth, with a dense and dense mature fibrous interstitial hyperplasia. The tumor cells are mainly small round cells with relatively uniform size, showing a lump or nest. It is buried in the stroma of hyperplastic fibers. In a few cases, tumor cells in some regions show different differentiation characteristics. Immunohistochemistry: 5 cases had epithelial expression (AE1/AE3 or EMA), 3 cases expressed NSE, 1 case expressed interstitial desmin, and 1 case expressed interstitial vimentin. In one case, electron microscopy was performed. Tensile fibrils and desmosomes were seen in the cells. Conclusions: (1) The age of onset of profibrotic small round cell tumors is relatively large and may occur in the abdominal cavity or outside the abdominal cavity; (2) The tumor has unique histological features, and most tumor cells are undifferentiated. The small epithelial cells are buried in the fibrotic mesenchyme. (3) In a few cases, the tumor cells may have different specific differentiation due to the different sites. The more mature and specific differentiation is a good prognosis marker; (4) The prognosis of the tumor is related to the site of its occurrence, its division activity and its differentiation.