论文部分内容阅读
目的研究具有血管周上皮样细胞分化的肿瘤(PEComas)的临床病理特点。方法回顾性分析、筛查外检工作中形态学上有 PEComas 特征的病例加做免疫组化后确定属于 PEComa 的病例,进行临床资料收集(尤其是有无结节性硬化表现)、病理形态学观察、免疫组织化学染色及部分病例的随访。结果共39例 PEComas 家族的病例。发生于肾30例,肝4例,肺1例,子宫及阔韧带1例,鼻腔1例,腹壁1例,腹膜后1例。未发现伴有 TSC 相关症状的病例。免疫组化检测提示 Melan-A是敏感性最高的抗体,表达率达95%(37/39),HMB45和 MiTF 分别是82%(32/39)和46%(18/39)。SMA82%(32/39)病例表达。Desmin 表达率26%(10/39)。S100表达率15%(6/39)。CD117仅10%病例灶性表达,且强度都较弱。没有病例表达 CK 和 CD34。20例病例随访资料显示,19例无病生存,1例带瘤存活。结论 PEComa 具有独特的形态学和免疫组化特征,需要与多种肿瘤鉴别,其严格的恶性标准尚需更多的病例和随访资料来证实。
Objective To investigate the clinicopathological features of tumors with perivascular epithelial cell differentiation (PEComas). Methods We retrospectively analyzed and screened the cases with morphological features of PEComas in the inspection work and identified the cases belonging to PEComa after immunohistochemistry. We collected the clinical data (especially the features of tuberous sclerosis), pathomorphology Observation, immunohistochemical staining and follow-up in some cases. Results A total of 39 cases of PEComas family. Occurred in the kidney in 30 cases, 4 cases of liver, lung in 1 case, uterus and broad ligament in 1 case, nasal cavity in 1 case, abdominal wall in 1 case, retroperitoneal in 1 case. No cases of TSC related symptoms were found. The results of immunohistochemistry indicated that Melan-A was the most sensitive antibody, with the expression rate reaching 95% (37/39), HMB45 and MiTF being 82% (32/39) and 46% (18/39) respectively. SMA 82% (32/39) case expression. Desmin expression rate of 26% (10/39). S100 expression rate of 15% (6/39). CD117 only focal expression of 10% cases, and the intensity is weak. No case of CK and CD34 cases of follow-up data showed 20 cases, 19 cases of disease-free survival, 1 case of tumor survival. Conclusions PEComa has unique morphological and immunohistochemical features that need to be identified with a wide range of tumors. Its strict malignancy criteria need more cases and follow-up data to confirm.