肾恶性上皮样血管平滑肌脂肪瘤一例并文献复习

发布时间：2018-05-31 01:30

本文选题：肾上皮样血管平滑肌脂肪瘤 + 临床特点　；参考：《山东大学》2017年硕士论文

【摘要】：目的:探讨肾上皮样血管平滑肌脂肪瘤的临床特点、病理特点、诊断方法、治疗及预后判断。提高对肾上皮样血管平滑肌脂肪瘤(EAML)的认识及诊疗水平。材料及方法:本次研究我们回顾性分析了山东省立医院泌尿外科于2017年3月3日收治的1例肾上皮样血管平滑肌脂肪瘤患者的临床资料。患者女,37岁,因"间断性肉眼血尿1月余"入院,当地医院B超检查示:右肾实性占位(Ca?)。CT检查示:右肾恶性肿瘤,肿瘤体积7.3cmx9.4cmx10.5cm,肿瘤形态不规则,动态增强扫描,动脉期肿瘤强化不明显,静脉期、肾实质期病灶明显强化,但强化程度较正常减低,病灶与正常肾脏对比轻度强化。病灶突破肾周脂肪囊,突破肾盂并挤压下腔静脉,边界欠清晰。腹膜后未见肿大淋巴结。患者于2017年3月6日在气管插管全麻下行腹腔镜根治性右肾切除术。检索中国知网和Pubmed等数据库中关于肾EAML的文献复习并讨论。结果:患者于2017年3月6日行腹腔镜下右肾根治性切除术。术后病理(病理号:S1702549):(右)肾恶性上皮样血管平滑肌脂肪瘤(11x9cm),输尿管切线及肾门血管未查见肿瘤。免疫组化染色黑色素瘤相关抗原(HMB-45)强阳性、MelanA阳性。患者术后7天后恢复良好,顺利出院,未行放、化疗等辅助治疗,随访至今暂未无复发及转移。结论及意义:肾血管平滑肌脂肪瘤(AML)是一个良性的病变过程。肾上皮样血管肌脂肪瘤(EAML)是肾脏血管肌脂肪瘤一种罕见的变异类型。2004年世界卫生组织男性和泌尿生殖系统肿瘤将它定义为"一种以上皮细胞为主的具有扩散的特征的潜在的恶性间质肿瘤"。EAML主要由类上皮细胞、梭形细胞和巨细胞组成,不包含或只有少量的脂肪瘤的组织,其影像学检查极易误诊为肾细胞癌或肉瘤。其治疗主要依靠手术切除,因病例罕见,研究较少,化疗效果尚无肯定结论。肾EAML的确诊主要依靠术后病理,尤其是免疫组织化学检查,特征表现为肿瘤细胞显示HMB-45、MelanA强阳性。约有1/3发展为恶性,恶性EAML以局部浸润以及淋巴结转移为主,预后总体较差。其治疗手段主要为手术切除,对于化疗药物及靶向治疗的研究很多,表柔比星、mTOR抑制剂依维莫司、吉西他滨等药物短期可能有效,但长期效果不明显。随着研究的不断深入,可以发现肾EAML的可能的活跃靶点,进而为肾EAML辅助治疗找到新方向。
[Abstract]:Objective: to investigate the clinical features, pathological features, diagnosis, treatment and prognosis of renal epithelioid angiomyolipoma. To improve the understanding and diagnosis and treatment of renal epithelioid angiomyolipoma (EAML). Materials and methods: we retrospectively analyzed the clinical data of a case of renal epithelioid angiomyolipoma treated in Urology Department of Shandong Provincial Hospital on March 3 2017. The patient was 37 years old. She was admitted to hospital because of "intermittent hematuria for more than one month." B-ultrasound examination in local hospital showed that the right renal solid space occupying place was Cajiao. Ct examination showed that the tumor volume was 7.3cmx9.4 cmx10.5cm, the shape of the tumor was irregular, and the dynamic contrast enhanced scan was performed, and the tumor size was 7.3cmx9.4 cmx10.5cm. The enhancement of tumor in arterial phase was not obvious, while in venous phase, renal parenchyma phase was obviously enhanced, but the enhancement degree was lower than that in normal stage, and the lesion was slightly enhanced compared with normal kidney. The lesion broke through the perirenal fat sac, broke through the renal pelvis and squeezed the inferior vena cava, and the boundary was unclear. No enlarged lymph nodes were seen behind the peritoneum. The patient underwent laparoscopic radical right nephrectomy under tracheal intubation and general anesthesia on March 6, 2017. To search for literature review and discussion on renal EAML in the databases of Chinese knowledge Network and Pubmed and so on. Results: laparoscopic radical nephrectomy was performed on March 6, 2017. Postoperative pathology (pathological number: S1702549% (right) malignant renal epithelioid angiomyolipoma (11 x 9 cm), ureteral tangent line and renal hilar vessel were not found. The melanoma associated antigen (HMB-45) was strongly positive and MelanA was positive. The patient recovered well 7 days after operation and was discharged smoothly, without radiotherapy, chemotherapy and other adjuvant treatment. There was no recurrence or metastasis during the follow-up. Conclusion and significance: renal angiomyolipoma (AMLA) is a benign process. Renal epithelioid angiomyolipoma (EAMLL) is a rare variant of renal angiomyolipoma. In 2004, WHO defined it as "a proliferative characteristic of epithelial cells in men and urogenital tumors." The underlying malignant interstitial neoplasms ". EAML mainly consists of epithelioid cells, A tissue consisting of spindle cells and giant cells that does not contain or contain only a small number of lipomas, and whose imaging findings are easily misdiagnosed as renal cell carcinoma or sarcoma. Its treatment mainly depends on surgical resection, because the case is rare, the research is less, the chemotherapeutic effect does not have the definite conclusion. The diagnosis of renal EAML mainly depends on postoperative pathology, especially immunohistochemical examination, which is characterized by tumor cells showing strong positive for HMB-45 and Melan A. About one third of the patients developed malignancy, and local invasion and lymph node metastasis were dominant in malignant EAML, and the prognosis was generally poor. Surgical resection is the main treatment method. There are many studies on chemotherapeutic drugs and targeted therapy. Evimoxus and gemcitabine inhibitors of epirubicin may be effective in the short term, but the long-term effect is not obvious. With the development of research, the possible active target of renal EAML can be found, and a new direction for renal EAML adjuvant therapy can be found.
【学位授予单位】：山东大学
【学位级别】：硕士
【学位授予年份】：2017
【分类号】：R737.11

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