原发性肾平滑肌肉瘤1例报告并文献复习

目的:探讨原发性肾平滑肌肉瘤的临床表现、影像学及病理特点、治疗方式和远期预后。方法:回顾性分析1例原发性肾平滑肌肉瘤患者的临床资料,该患者为70岁女性,主要表现为腰部疼痛症状。双肾彩超和CT均提示左肾占位性病变,密度不均一。予以左肾根治性切除术。术后随访。结果:术后病理回报(左肾脏)高度可疑为平滑肌肉瘤(直径8 cm);左侧肾盂、输尿管、肾门血管断端未见癌;腹主动脉旁淋巴结3枚,未见癌转移(0/3)。HE及免疫组化结果支持平滑肌肉瘤(直径8 cm),Desmin(++),Caldesmon(+++),CD10(-),Ki67(40%～60%),p53(弥漫++)。患者术后2个月未见局部肿瘤复发和肺转移。结论:原发性肾平滑肌肉瘤在临床上十分罕见,其恶性程度高,预后差。临床表现和影像学检查无特异性,术后病理诊断为其金标准。手术根治切除为目前主要治疗方法,辅助性放化疗当前仍存在争议。

Primary leiomyosarcoma of the kidney:A case report and literature review

Objective:To investigate the clinical manifestation,imaging medicine,pathological characteristics,therapeutic methods and long-term prognosis of primary leiomyosarcoma of the kidney.Methods:Retrospectively analyse clinical data of a case of primary leiomyosarcoma of the kidney.This patient was 70 years old female.The main manifestation was lumbar pain symptom.Bilateral kidney colour doppler ultrasound and CT suggested that the left kidney occupied lesions and the density was not uniform.Treatment was radical excision of left kidney.Then postoperative follow-up was completed.Results:Postoperative pathology showed left kidney was highly suspected as leiomyosarcoma(diameter with 8 cm).No cancer was found at the end of the vessel of renal pelvis,ureter and hilum.3 lymph nodes adjacent to abdominal aorta had no metastasis（0/3).HE and immunohistochemical results supported leiomyosarcoma,Desmin（++），Caldesmon（+++），CD10（-），Ki67（40%~60%），p53（suffuse ++）.Local tumor recurrence and lung metastasis were not found in the patients 2 months after operation.Conclusion:Primary leiomyosarcoma of the kidney is rare clinically.Its malignant degree is high and its prognosis is poor.There is no specificity in clinical manifestation and imaging examination.Postoperative pathological diagnosis is the gold standard.Radical resection is the main treatment at present,and adjuvant radiotherapy and chemotherapy are still controversial.