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[目的]探讨肾上皮样血管平滑肌脂肪瘤(EAML)的临床特点、诊治及预后。[方法]回顾性分析8例肾EAML患者的临床特点、诊治及预后的临床资料。[结果]8例患者均行了手术治疗,术后病理及免疫组化符合肾EAML诊断。随访时间为5~98个月,平均47个月,无瘤生存6例,另2例生存时间分别为9个月和21个月。[结论]EAML是具有恶性潜能的血管平滑肌脂肪瘤特殊亚型。治疗方案首选手术切除,术后需严密随访,由于其对放化疗及靶向治疗不敏感,一旦肿瘤复发转移,预后差。
[Objective] To investigate the clinical features, diagnosis, treatment and prognosis of renal epithelioid angiomyolipoma (EAML). [Methods] The clinical features, diagnosis, treatment and prognosis of 8 patients with renal EAML were retrospectively analyzed. [Results] All 8 patients underwent surgical treatment. Postoperative pathology and immunohistochemistry were consistent with renal EAML diagnosis. Follow-up time was 5 to 98 months, an average of 47 months, 6 patients survived without disease, and the other 2 patients survived 9 months and 21 months respectively. [Conclusion] EAML is a special subtype of malignant potential angiomyolipoma. The first choice of surgical resection, follow-up after surgery, because of its radiotherapy and chemotherapy and targeted therapy is not sensitive, once the recurrence and metastasis of the tumor, the prognosis is poor.