摘要
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 invewstigate the clinicopathologic features of tumors showing perivascular epithelioid cell differentiation （PEComas）. Methods The clinicopathologic data of 39 cases pf angiomyolipoma （AML）, 17 males and 22 females, with the primary focus in the kidney on 30 cases, in the liver in 4 cases, in the lung, uterus and broad ligament, abdominal wall , retroperitoneum, and nasal cavity in 1 case respectively, were analyzed. Immunohistochemistry （HIC）was used to detect the expression of pan-cytokeratin （CK）, S-100 protein, smooth muscle actin （SMA）, desmin, vimentin, HMB45, MelanA, microphthalmia transcription factor （ MiTF）, CD117, and CD34in the specimens of the tumors obtained during operation. Twenty patients were followed up. Results Pathological examination showed branched capillaries or arterioles, often thick-walled similar to those in the renal cell carcinoma, and the cancerous cells consisting of the mixture of epithelial cells and spindle cells. HIC showed that the expression rates of Melan-A,HMB45, MITF,SMA, desmin , S-100 protein, vimentin, CD117, CK, and CD34 were 95% （37/ 39）, 72% （32/39）, 46% （18/39）, 82% （32/39）, 27% （10/39）, 15% （6/39）, 82% （32/39）, 10% （ 4/39）, 0, and 0 respectively. Clinical follow-up showed 1 patient alive with tumor, and 19 alive free from disease. Conclusion PEComas have distinctive morphological and immunohistochemical features.
出处
《中华医学杂志》
CAS
CSCD
北大核心
2007年第41期2909-2913,共5页
National Medical Journal of China
关键词
上皮样细胞
细胞分化
肿瘤
Epithelioid cell
Cell differentiation
Neoplasms
