摘要
目的探讨肾脏神经内分泌肿瘤的生物学行为、临床病理特征,提高对该类肿瘤的认识和诊治水平。方法分析1999~2010年在中山大学肿瘤医院收治的3例肾脏神经内分泌肿瘤临床病理特点并结合文献复习,讨论该肿瘤的组织来源、病理和免疫组化特征、诊治及预后。结果本研究中3例患者均表现为腰部肿物,无特异性的类癌综合征表现。2例为男性,2例发病年龄〈40岁,3例均行手术,术后病理2例为类癌,1例肾母细胞瘤伴神经内分泌分化,3例免疫组化染色肿瘤细胞CK及Vim（＋）、2例Syn（＋）、2例NSE（＋）,1例CgA（＋）。术后1例行化疗,1例行生物治疗联合放疗,1例未行治疗。随访时间6个月-6年,其中1例死于肿瘤转移,1例失访,1例无病生存至今。结论肾脏神经内分泌肿瘤是一种比较少见的异质性高的恶性肿瘤,有独特的病理特征,临床表现主要为腹部肿块、急性腰痛和血尿,确诊依靠病理检查及免疫组化,疾病预后差。对于早期类癌,肾切除术能根治,对于进展期肿瘤,预后仍然较差,强调多学科治疗。
Objective To study the biological and clinicopathological characteristics of neuroendocrine tumors of the kidney（KNETs） for improving the diagnosis and treatment of this diseases.Methods The pathological and clinical features of 3 KNET cases treated in Sun Yat-sen University Cancer Center between 1999 and 2010 were summarized,and the the histogenesis,pathological and immunohistochemical characteristics,diagnosis and prognosis of KNETs were analyzed with review of all reported cases worldwide.Results All the 3 patients had waist masses but without clinical manifestations of carcinoid syndrome,and underwent resection of the tumors.The postoperative pathological diagnosis was carcinoid carcinoma in 2 patients and Wilms＇ tumor with neuroendocrine differenciation in 1 patient.Immunohistochemical examination showed that the tumors were positively stained for cytokeratin and vimentin;positivity for neuron-specific enolase and synaptophysin was found in 2 cases,and chromogranin positivity in 1 case.After the operation,1 patient received chemotherapy,1 had biotherapy and radiotherapy,and 1 received no further treatment.During the follow-up from 6 months to 6 years,1 patient died of tumor metastasis,1 was lost to follow-up,and 1 showed no recurrence until now.Conclusions KNETs has specific pathological characteristics.Abdominal masses,acute loin pain and hematuria are the most common symptoms.A definite diagnosis relies on pathology and immunohistochemistry.For early carcinoid carcinoma,surgical resection is curable,but in advanced cases,the prognosis is poor and comprehensive therapy is recommended.
出处
《南方医科大学学报》
CAS
CSCD
北大核心
2011年第3期548-550,共3页
Journal of Southern Medical University
关键词
神经内分泌肿瘤
类癌
肾脏肿瘤
neuroendocrine tumor
carcinoid carcinoma
kidney neoplasms
