上皮样血管平滑肌脂肪瘤7例临床病理分析  

Epithelioid angiomyolipomas: a clinicopathological analysis of 7 cases

作　　者：徐园园[1,2] 胡维维[1,2] 张良运[1,2] 李广民[1,2] 

机构地区：[1]中山大学附属佛山医院 [2]佛山市第一人民医院病理科,佛山528000

出　　处：《临床与实验病理学杂志》2015年第2期161-163,168,共4页Chinese Journal of Clinical and Experimental Pathology

摘　　要：目的：回顾分析上皮样血管平滑肌脂肪瘤（ epithelioid angiomyolipoma, EAML）的形态学特点、免疫表型、诊断及鉴别诊断、临床特点及预后,加深对EAML的认识,提高诊断的准确性。方法观察7例EAML的病理形态学、免疫表型及临床特征。结果7例EAML形态相似,肿瘤细胞体积大,呈卵圆形、梭形或多边形,胞质丰富,嗜酸性,细胞核大,大小不一,核仁明显,可见多核及巨核瘤细胞,有少数核分裂象,呈巢、片状分布,部分围绕血管呈袖套状。免疫表型：肿瘤细胞HMB-45、Melan-A、vim-entin、SMA均弥漫阳性,EMA、CK、CD10均阴性。7例EAML均行肿瘤根治性手术或肿瘤切除术,术后随访3~56个月,除1例因有高度恶性十二指肠间质瘤病史于3个月后死于恶性间质瘤进展外,其余6例均健在,多次复查B超、CT等均无复发、转移。结论 EAML是具有恶性潜能的间叶源性肿瘤,病理形态学上易误诊为恶性肿瘤,免疫组化对鉴别诊断有重要的意义,目前手术治疗是主要方法,有复发或转移可能。Purpose To analyze and summarize morphological characteristics, the immune phenotype, diagnosis and differential diag-nosis, clinical features and prognosis of epithelioid angiomyolipomas retrospectively, to deepen understanding of EAML and improve the diagnostic accuracy. Methods The pathological morphology was observed and immunohistochemistry of phenotypic characteristics were analyzed in 7 cases of epithelioid angiomyolipomas. Results Seven cases of epithelioid angiomyolipomas had similar morphology：larger tumor cells, ovoid, fusiform or polygonal, with abundant and eosinophilic cytoplasm, a big nucleus of different size with obvious nucleoli, polynuclear and gaint tumor cells, with a few mitotic figures. The tumor cells arranged in nests or sheets, cuff-shaped around the blood vessels. Immunohistochemistry showed that the tumor cells were positive for HMB-45, Melan-A, vimentin and SMA, but negative for EMA, CK, and CD10. All of them underwent radical surgery or surgical resection, and were followed up for 3~56 months after operation. 6 cases were alive, without recurrence and metastasis, while 1 case died of gastrointestinal stromal sarcomas 3 months later. Conclusion Epithelioid angiomyolipomas belong to mesenchymal tumors with malignant potential, pathological morphology would be easily misdiagnosed as malignant tumors. Immunohistochemistry plays an important role in the differential diagnosis. Surgical treatment is the main method at present, with possibility of recurrence or metastasis.

关 键 词：上皮样血管平滑肌脂肪瘤 病理学 免疫组织化学 诊断 

分 类 号：R730.262[医药卫生—肿瘤]