摘要目的 总结肾小细胞性神经内分泌癌(简称小细胞癌)的临床病理学特征,分析其诊断及鉴别诊断依据.方法 收集1999年至2010年间解放军总医院病理科诊断的肾小细胞癌12例患者的临床资料,采用HE及免疫组织化学EnVision法进行病理学观察,并结合临床特征进行分析.结果 12例中6例为肾原发性小细胞癌,其中2例发生于肾实质,4例发生于肾盂；胸部影像学检查双肺均未发现占位性病变,肾均为单发肿瘤；5例行根治性肾切除术；大体检查见4例肿瘤位于肾盂,1例位于肾实质.其余6例为肺小细胞癌肾转移,其中4例为肺小细胞癌治疗过程中检查发现,2例以腰痛为首发症状就诊,同时发现肺部与肾的肿瘤；6例均行穿刺活检.显微镜下12例均呈典型的小细胞癌形态,坏死广泛.4例原发于肾盂的病例中可见部分尿路上皮癌成分.免疫组织化学染色显示12例肿瘤细胞均呈CK、突触素、CD56阳性,6例转移性病例和4例原发性病例甲状腺转录因子1阳性.6例肾原发性小细胞癌患者中2例分别于术后4及9个月死亡,2例失访,2例存活(分别随访25及138个月)；6例转移性患者5例死亡(随访3～8个月),1例随访1个月仍存活.结论 肾小细胞癌有原发和转移两种情况,虽然罕见,但肾可以原发小细胞癌,肾盂和肾实质均可发生,诊断主要依靠病理学检查,甲状腺转录因子1在鉴别原发与转移性肿瘤时参考意义不大,鉴别诊断需要结合临床病史、胸部影像学检查和形态学有无合并尿路上皮癌成分等来综合判定.

abstractsObjective To study the clinicopathologic features and histologic differential diagnosis of small cell neuroendocrine carcinoma (SmCC) of kidney.Methods The clinicopathologic features of 12 cases of SmCC of kidney encountered during the period from 1999 to 2010 were retrospectively reviewed.Results Six eases of primary and 6 cases of metastatic SmCC involving kidney were identified.Amongst the primary renal SmCC,2 were located in renal parenchyma and 4 in renal pelvis.Chest X-ray showed negative findings.Five of them underwent radical nephrectomy. On gross examination,the tumor was located centrally around the renal pelvis in 4 cases and peripherally in renal parenchyma in 1 case.On the other hand,4 of the 6 cases of metastatic SmCC were discovered during therapy for pulmonary SmCC.Two of these patients presented with abdominal pain and gross hematuria,with lung and renal tumor masses identified simultaneously.The diagnosis of all the 6 cases of metastatic SmCC was confirmed by fine needle aspiration biopsy.Microscopically,pure SmCC was demonstrated in the 2 cases of primary renal parenchymal SmCC and 6 cases of metastatic SmCC.The 4 primary renal pelvic SmCC coexisted with urothelial carcinoma component.On immunohistochemical study,all cases were positive for cytokeratin,synaptophysin and CD56.All metastatic cases and 4 primary cases were also positive for TTF-1.Of six patients with primary SmCC two died 4 and 9 months after operation,and two were alive with a follow-up of 25 and 138 months,respectively.Five of six cases with metastatic SmCC died 3-8 months after diagnosis.The other 3 cases were failed to follow-up.Conclusions Both primary and metastatic SmCC can be found in the kidney.Although rare,primary SmCC is located either in renal parenchyma or in pelvis.The diagnosis of SmCC relies on morphologic examination and immunohistochemical study.TTF-1 immunostaining cannot reliably distinguish primary from metastatic SmCC in kidney.Correlation with clinicoradiologie findings and demonstration of coexisting urothelial carcinoma component ( if any) is helpful in delineation of the tumor origin.