肾脏原发性神经内分泌肿瘤五例报告并文献复习  

Primary renal neuroendocrine tumors：report of five cases and review of the literature

作　　者：熊轶 张古田 樊祥山 史炯 努尔艾力·排尔哈提 何炜[3] 甘卫东 李笑弓 郭宏骞 

机构地区：[1]南京鼓楼医院泌尿外科,210008 [2]南京鼓楼医院病理科,210008 [3]安徽省巢湖市第二人民医院泌尿外科

出　　处：《中华泌尿外科杂志》2016年第2期85-89,共5页Chinese Journal of Urology

摘　　要：目的：分析肾脏原发性神经内分泌肿瘤的诊断、治疗和预后特点，以提高对该肿瘤的认识。方法回顾性分析2008年1月至2015年6月住院治疗的5例原发性肾脏神经内分泌肿瘤的临床资料。男1例，女4例。年龄40-73岁，中位年龄48岁。肿瘤均位于左侧肾脏，直径4-9 cm，平均6．5 cm。临床表现为肉眼血尿1例，腹痛1例，健康体检发现3例。肾脏超声造影检查4例，3例提示中低回声团块，1例提示高回声团块。术前CT检查5例，均见不规则形团块状影，密度欠均匀，平扫见钙化灶3例，增强见明显不均匀强化3例，中度不均匀强化1例。结果5例均行手术治疗，3例行肾根治性切除术，2例行肾部分切除术。病理检查：3例肿瘤细胞呈巢团状或缎带样排列，浸润性生长，无或偶见核分裂象，无坏死，符合肾类癌，其中1例肾门淋巴结3／4枚见肿瘤细胞。1例肿瘤细胞呈缎带样排列，部分细胞核呈圆形，异型性不显著，核分裂象少见，符合肾脏非典型类癌，肾门淋巴结2／2枚见肿瘤细胞。1例肿瘤组织内形态一致的中等大小细胞呈巢团状排列，有坏死，核质比大，异型明显，核分裂象多见，符合肾小细胞癌。5例免疫组化染色检查示突触素（ Syn）、嗜铬蛋白A（ CgA）阳性。术后随访时间4-78个月，中位随访时间11个月。3例肾类癌中，1例在行开放肾部分切除术后78个月局部复发，后行根治性手术；另2例肾部分切除术后随访8-27个月未见复发或转移。1例肾非典型类癌行肾根治性切除术，术后随访24个月未见复发或转移。1例肾小细胞癌行根治性肾切除术＋肝脏转移肿瘤射频消融术，术后约11个月死于多器官功能衰竭。结论肾脏原发性神经内分泌肿瘤临床罕见，可表现为类癌、非典型类癌或小细胞癌，其临床表现、病理特征及预后差异较大；原发性肾类癌可手术治疗，预后尚�Objective To analyze the features of diagnosis, treatment and prognosis of primary renal neuroendocrine tumors, and to improve the understanding of primary renal neuroendocrine tumors. Methods From January 2008 to June 2015, 5 cases of primary renal neuroendocrine tumors were hospitalized and their data was analyzed retrospectively, with 1 male 4 females, aged 40-73 years with the middle age of 48 years.Tumors were all located in the left kidney, whose diameters arranged from 4 to 9 cm, with an average of 6.5cm.One case presented with hematuria, one case was identified because of abdominal pain, and the other three cases were identified via physical examination.Four cases underwent a renal contrast-enhanced ultrasound, which indicated a medium-hypoechoic mass in three cases and a hyperechoic mass in one case.All five cases underwent CT scan, presenting irregularly shape and density. Calcification was found in three cases on plain scanning.Significantly heterogeneous enhancement was found in three cases and moderate heterogeneous enhancement was found in one case on enhanced scanning. Results All five cases underwent operations, with two cases undergoing radical nephrectomy and three cases undergoing partial nephrectomy.Pathological examination showed three cases of tumor cells arranged in a nest slug or ribbon-like infiltrative growth, with no or rare mitosis and no necrosis, which is consistent with renal carcinoids.Three out of 4 renal hilar lymph nodes containing tumor cells were identified in one case. The tumor cells in another case exhibited a ribbon-like arrangement, with some round nuclei, no significant atypia, and rare mitosis, which is consistent with renal atypical carcinoid.Two out of two renal hilar lymph nodes containing tumor cells were identified in this case. The tumor tissue of one case showed morphologically uniform, medium-sized cells arranged in nest slug form with necrosis, a high nuclear cytoplasm ratio, an obvious allotype and frequent mitosis, which is consistent with renal small cell ca

关 键 词：肾 神经内分泌肿瘤 类癌 非典型类癌 小细胞癌 

分 类 号：R737.11[医药卫生—肿瘤]