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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 analyze the rare renal primary neuroendocrine tumors (including carcinoid and small cell carcinoma) and to investigate the clinicopathological characteristics and prognosis. Methods The clinical data of 6 patients with renal neuroendocrine tumors were collected. The pathological changes were observed by HE and immunohistochemical staining, and the clinical features were analyzed. Results Of the 6 cases, 4 were carcinoid, ranging in age from 31 to 46 years; 2 were small cell carcinomas, aged 28 and 60 years. 6 cases of tumors are located in the right kidney. Of the 4 carcinoid patients, 3 were found by physical examination and 1 patient with horseshide kidney was the first manifestation of the right frontal lobe metastasis. The clinical manifestations of 2 patients with small cell carcinoma were low back pain with gross hematuria and no carcinoid in 6 patients Syndrome performance. 4 cases of carcinoid tumor diameter of 2.5 ~ 10 cm (average 6.6 cm), clear boundary, no significant necrosis. Two cases of small cell carcinoma tumors were 21 cm and 5.5 cm in diameter, with unclear boundaries and many necrotic sections. Microscopically, carcinoid cells were arranged in the shape of beams, ribbons, adenoids or astrocytes. The mitotic figures were either rare or even necrotic. Small cell carcinoma showed no organ-like arrangement with small cytoplasm, large nucleus, Sexually obvious, mitotic common, necrosis widespread. Carcinoid in 1 case was diagnosed with right frontal lobe and retroperitoneal lymph node metastasis, the other 3 cases no lymph node and distant metastasis; 1 case of small cell carcinoma with IVC tumor thrombus formation, the other 1 case With retroperitoneal lymph nodes and liver, brain and other distant metastasis. One case of carcinoid tumor with horseshoe kidney and brain metastasis was followed up for 132 months with tumor survival, and the other 3 cases were followed up for 24-36 months without tumor. Small cell carcinoma in 1 case of puncture diagnosis of lost cases, 1 case 9 months after the death of multiple organ failure. Conclusions Although rare, kidney can be a primary neuroendocrine tumor with carcinoid and small cell carcinoma. The histological morphology and the prognosis are different, and the prognosis of small cell carcinoma is poor. The prognosis is relatively good.