肾平滑肌肉瘤1例报告并文献复习

目的：探讨肾平滑肌肉瘤（LMSK）的临床特点和诊治方法.方法：回顾性分析1例LMSK患者的临床资料,并结合文献复习讨论LMSK的临床表现、病理特点、诊治方法和预后.患者为女性,主要表现为腰部阵发性疼痛8天,B超和CT提示左肾占位性病变.行根治性肾切除术.结果：病理检查肿瘤直径6 cm,光镜下见肿瘤细胞主要为平行束状或交织束状排列的嗜酸性梭形细胞,胞核钝圆,胞浆嗜酸性.免疫组织化学染色α-平滑肌肌动蛋白（α-SMA）和结蛋白（Des）阳性,细胞角蛋白（CK）和黑色素瘤抗体（HMB-45）阴性.TNM分期为T2b N0M0,分级为G2.患者术后5个月死于局部肿瘤复发和肺转移.结论：LMSK临床少见,恶性程度高,预后差.临床表现和影像学检查无特异性表现,通常为术后病理检查确诊.手术为主要治疗手段,辅助化疗和放疗效果尚有争议.

Leiomyosarcoma of the Kidney(Report of 1 Case and Review of the Literature)

Objective： To investigate the clinical features of leiomyosarcoma of the kidney （LMSK） and the methods of diagnosis and treatment. Metlhods：1 case of LMSK confirmed by surgery and pathology was analyzed. and the clinical presentations, pathologic characteristics, the diagnosis, treatment and prognosis of LMSK were dis- cussed with review of the literature. The patient was a female,presented with complaint of intermittent lumbago in left side for 8 days. Uitrasound-B and CT scan of the abdomen revealed a space-occupying lesion arising from the left kidney. Results：The patient underwent radical nephrectomy. The tumor was 6 cm in diameter, The pathology showed alternating fascicles of spindle cells with blunt-ended non-tapering nuclei and eosinophilic cytoplasm. Im- munohistochemistry showed tumor cells with diffuse cytoplasmic positivity for α-smooth muscle actin （α-SMA） and desmin （Des）. They were negative for cytokeratin（CK） and HMB-45. By TNM staging： T2b No M0, Gz, stage： Ⅱ A. The patient died of local recurrence and lung metastasis 5 months after operation. Conclusions. LMSK is an extremely rare and highly malignant with poor prognosis, clinical presentations and radiologic features are nonspe- cific and diagnosis is usually made postoperatively. Surgical excision being the primary treatment, the efficacy of ad- juvant chemotherapy and irradiation remains debatable.