巨大原发性肾平滑肌肉瘤1例报告和文献复习

Giant primary renal leiomyosarcoma: a case report and literature review

目的:探讨原发性肾平滑肌肉瘤的临床特点、影像学表现、病理学特点、治疗方法和预后。方法:回顾性分析2014年收治的1例原发性肾平滑肌肉瘤患者的临床资料,并结合相关文献复习讨论原发性肾平滑肌肉瘤的临床特点、影像学表现、病理学特点、治疗方法和预后。患者,女,70岁。主要表现为全程肉眼血尿1周,伴腰痛、发热及腹部包块。CT和B超检查提示左肾实质性占位性病变,考虑左肾癌,肿瘤直径约15 cm。术前肿瘤分期为T4N0M0,行左肾根治切除治疗。结果:术后病理检查为左肾平滑肌肉瘤,肿瘤大小为17 cm×17 cm×12 cm。术后8个月患者死亡。结论:肾平滑肌肉瘤临床比较罕见,临床症状有肉眼血尿、腰腹部肿块、腰痛、贫血、发热等,和常见的肾癌一样,无特异性。影像学检查也无特异性。确诊要依据病理检查。外科手术为主要治疗手段,放疗、化疗、靶向治疗等辅助治疗效果尚不明确。

Objective： To study the clinical features,imaging findings,pathological characteristics,treatment and prognosis of primary renal leiomyosarcoma（ RLS）. Methods： Retrospective analysis was conducted on the clinical data of a case of primary RLS admitted to our hospital in 2014,and through the review of relevant literature,a discussion of the primary RLS was held in terms of its clinical features,imagings,pathologic characteristics,treatment and prognosis. The patient was a female,70 years old. The main manifestation was the whole course of macroscopic hematuria for 1 week,with low back pain,fever and abdominal mass. CT and B ultrasound examination showed left renal parenchymal lesions,indicating the left renal cell carcinoma,with diameter of about 15 cm. Preoperative tumor staging was T4N0M0,and radical resection of the left kidney was performed. Results： The postoperative pathological examination manifested that the tumor size was 17 ＊ 17 ＊ 12 cm. 8 months after the surgery,the patient died. Conclusion： Leiomyosarcoma of the kidney is clinically rare. The clinical symptoms include macroscopic hematuria,abdominal mass,low back pain,anemia,fever,etc.Just like common renal carcinoma,it is not specific. The diagnosis should be based on the pathological examination. Surgical operation is the main treatment means. The effect of radiotherapy,chemotherapy and targeted therapy is not clear.