成人原发性肾肉瘤9例临床诊疗分析

背景与目的：肾原发性肉瘤少见，临床诊断较困难，探讨原发于肾脏肉瘤的临床组织发生、病理学特点、诊断、治疗及预后诸因素。方法：总结我院收治9例原发性肾肉瘤患者临床资料，术前行B超、CT或MRI检查，行病肾根治性切除术或肿瘤切除术。对肾肉瘤标本进行常规HE、组织化学及免疫组织化学染色观察。结果：9例显微镜下表现、组织化学及免疫组织化学染色分别显示恶性纤维组织细胞瘤、平滑肌肉瘤、脂肪肉瘤、粘液肉瘤、肾滑膜肉瘤的特点。由病理确诊，术前仅一例怀疑肉瘤。结论：原发性肾肉瘤主要表现为患侧腰疼，肿块：影像学检查不易与肾癌及肾错构瘤相鉴别，但依靠病理检查，配以组织化学染色和免疫组织化学染色可确诊：该肿瘤恶性度高，且位置深，一般发现已属晚期，故预后甚差。化疗、放疗效果争议较大，手术切除病肾是一可行方法。

The diagnosis and treatment of adult primary renal sarcoma : 9 cases report and literature review

Background and purpose:To investigate the clinico- pathological characteristics, prognosis and diagnosis of primary renal sarcoma that is a rare, highly malignant tumor with poor prognosis and survival. Methods:9 cases of renal sarcoma in our hospital were reviewed and analyzed. Nephrectomy was the main treatment for the patients. All the specimens were stained with histochemicial and immunohistochemical method, correlated literatures were also reviewed.Results:The distributions of pathology were malignant fibrous histiocytoma leiomyosarcoma, liposarcoma, myxosarcoma synoviosarcoma. Immunohistiochemical test was essential for the diagnosis of renal sarcoma.Conclusions:Pain and palpable mass were the common symptoms. Early radical surgery remains the optimal treatment, the role of chemotherapy and radiotherapy in the treatment modalities of the disease is still controversial.