肾血管平滑肌脂肪瘤临床病理特点分析

目的通过对肾血管平滑肌脂肪瘤（AML）临床病理特点观察及文献复习，旨在探讨肾AML的诊断及鉴别诊断。方法 对6例肾AML进行临床病理学及免疫组化观察、随访，并复习相关文献。结果 6例肾AML均属经典型，均位于右肾。2例混有上皮样细胞成分，2例多发性，2例出血及坏死。镜检：肿瘤由不同比例的厚壁血管、平滑肌和脂肪组织组成，平滑肌细胞有一定异型性，上皮样细胞胞质丰富，透明／微嗜酸性；核大深染，核仁大而明显，嗜酸性，可见多核瘤巨细胞；免疫组化：Vimentin、HMB45、MelanA、SMA均阳性，CK-pan、CD34阴性，ER、PR、P53、S100、Ki-67不同程度阳性。结论肾AML是良性肿瘤，但当肿瘤组织巨大、出现上皮样细胞成分伴有凝固性坏死、核异型性明显、核增殖活性高及核分裂像多时，应高度考虑为恶变，术后应长期密切随访。

Clinical pathological characteristics of renal angioleimyolipoma

Objective To discuss the diagnosis and differential diagnosis of renal angioleimyolipoma （AML）by observing its clinical pathological features and reviewing literatures. Methods Observation and follow up of clinical pathology and immunohistochemistry were made among 6 cases of renal AML, and relative literatures were reviewed. Results All these 6 cases of renal AML were the classical type with the AML located in the right kidneys. Two cases had epithelioid cell components;2 ones were multiple ;2 ones had hemorrhage and necrosis. Under the microscope, the tumor consisted of thick wall vessels, smooth muscle, and fat tissues of different proportion. The smooth muscle had certain heteromorphism. The epithelioid cells had rich cytoplasm which was transparent/slightly eosinophilic. The karyomegaly was deeply dyed and the nucleolus was big, clear, and eosinophilic, and multinucleated tumor giant ceils could be observed. Immunohistochemical staining indicated that Vimentin, HMIM5, MelanA, and SMA were all positive;CK- pan and CD34 were negative; ER, PR, P53, S100, and Ki67 were positive in different degrees. Conclusion Renal AML is a benign tumor, but malignant transformation should be considered when the tumor tissue is huge, epithelioid cell components accompanied with coagulation necrosis, obvious nuclear atyia, active nuclear proliferation and nuclear fission. Long - term and close follow up should be carried out after the operation.