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目的:探讨肾原发性神经内分泌癌(NECK)的临床病理特点,提高对该疾病的诊治水平。方法:回顾分析2000年1月~2015年11月期间诊治的10例病理诊断为NECK患者的临床病理资料。归纳总结该类肿瘤的临床特点、病理学特点、诊疗手段及临床预后。结果:10例患者病理诊断均为NECK,其中男2例,女8例,平均年龄40.1(22~70)岁。左肾2例,右肾8例。肿瘤最大径平均7.35(2.5~21.0)cm。TNM分期:T1N0M02例,T2N0M03例,T3N0M04例,T3N1M01例。10例患者均行手术治疗。平均随访21.2(1~75)个月,1例失访,2例患者术后发生远处转移,2例死亡。结论:NECK临床罕见,诊断依赖病理组织学及免疫组化检查,治疗以手术为主,辅以化疗,其中类癌恶性度较低,预后相对较好,其余类型恶性度高,发展迅速,预后较差。
Objective: To investigate the clinicopathological features of primary neuroendocrine carcinoma (NECK) and to improve the diagnosis and treatment of this disease. Methods: The clinicopathological data of 10 patients diagnosed as NECK from January 2000 to November 2015 were retrospectively analyzed. Summarize the clinical features of the tumor, pathological features, diagnosis and treatment methods and clinical prognosis. Results: The pathological diagnosis of 10 patients were all NECK, including 2 males and 8 females, with an average age of 40.1 (22-70) years. 2 cases of left kidney, 8 cases of right kidney. The largest diameter of tumor average 7.35 (2.5 ~ 21.0) cm. TNM staging: T1N0M02 cases, T2N0M03 cases, T3N0M04 cases, T3N1M01 cases. All 10 patients underwent surgical treatment. The patients were followed up for an average of 21.2 months (range, 1 to 75) months. One patient lost follow-up. Two patients had distant metastasis and two patients died. Conclusion: NECK is rare in clinic. The diagnosis depends on histopathology and immunohistochemistry. Surgical treatment is the mainstay of the treatment. Chemotherapy is used. The malignancy of carcinoid carcinoids is relatively low. The prognosis is relatively good. The remaining types of malignancy are high and develop rapidly. Prognosis Poor.