肾原发性神经内分泌肿瘤6例临床病理分析

Clinicopathological characteristics of primary renal neuroendocrine neoplasms:an analysis of six cases

目的分析罕见的肾原发性神经内分泌肿瘤(包括类癌和小细胞癌),探讨其临床病理特征及预后。方法收集6例肾神经内分泌肿瘤患者的临床资料,采用HE及免疫组化染色进行病理学观察,并结合临床特征进行分析。结果 6例中4例为类癌,年龄31～46岁;2例为小细胞癌,年龄28岁和60岁。6例肿瘤均位于右肾。4例类癌患者中3例为体检偶然发现,1例伴马蹄肾患者以右脑额叶转移为首发表现;2例小细胞癌患者临床表现为腰痛伴肉眼血尿;6例临床均无类癌综合征表现。4例类癌肿瘤直径为2.5～10 cm(平均6.6 cm),边界清楚,无明显坏死。2例小细胞癌肿瘤直径分别为21 cm和5.5 cm,边界不清,切面见多处坏死。镜下类癌瘤细胞排列呈梁状、缎带状、腺样或菊形团样,核分裂象无或偶见,无坏死;小细胞癌均未见器官样排列,细胞质少、核大,异型性明显,核分裂象多见,坏死广泛。类癌中1例诊断时即伴有右脑额叶及腹膜后淋巴结转移,另3例未见淋巴结及远处转移;小细胞癌诊断时1例伴有下腔静脉癌栓形成,另1例伴有腹膜后淋巴结及肝、脑等远处转移。类癌中1例伴马蹄肾并脑转移的患者随访132个月仍带瘤生存,另3例随访24～36个月无瘤生存。小细胞癌中1例穿刺诊断病例失访,1例术后9个月死于多器官功能衰竭。结论此瘤虽然罕见,但肾可以原发神经内分泌肿瘤,表现为类癌和小细胞癌特征,二者组织学形态明显不同,预后亦不同,小细胞癌预后很差,而类癌即使发生转移预后也相对较好。

Objective To report 6 cases of primary neuroendocrine neoplasms（NENs） of the kidney,including carcinoid tumour and small cell carcinoma（SmCC）,and to investigate their clinicpathological features and biological behaviour.Methods The clinicopathological features were reviewed in all 6 renal NENs diagnosed during a 11-year period.Results Four carcinoids with age of 31-46 years and two SCC with age of 28 and 60 years were identified from all the kidney tumours.All the six tumors located in right kidney.Three of the four carcinoids were found occasionally,and the other one with horseshoe shape presented with tumor metastasis to right frontal lobe of brain.2 SmCC patients presented with abdominal pain and gross haematuria.No patient had carcinoid syndrome.The size of the four carcinoids was 2.5~10 cm（mean 6.6 cm）and two SCC was 21 cm and 5.5 cm,respectively.Four cases of carcinoid were all well circumscribed without necrosis.By contrast three SmCC had extensive tumour necrosis with infiltrating margin.Microscopically,the tumor cells were trabecular,acinar or rossette-like in four carcinoids,with no or rare mitosis.However,two SmCC had extensive tumour necrosis and numerous mitosis,without organoid arrangements.Three of 4 carcinoid tumours were confined to the kidney,but one patient with right frontal lobe metastasis before the diagnosis of renal tumor.One SmCC with tumor embolization in inferior caval vein died 4 months after operation;the other SmCC with multiple metastases was failed to follow-up after diagnosis.Three of four carcinoid patients were disease-free with a follow-up of 24-36 months,and the other one was alive with tumor for 132 months.Conclusions Various NETs occasionally occur in the kidney,including carcinoid tumor and SmCC.Carcinoid and SmCC have different histologic features and prognosis.SmCC is associated with poor clinical outcomes,but patients with carcinoid can have a prolonged clinical course,even in the presence of metastasis.