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| 肾集合管癌的临床诊治经验及预后分析 |
| 杨启维1△,杨炜1△,徐梦微2,叶剑青1,干思舜1,潘秀武1,王磊1,田毅君1,曹建伟1,崔心刚1* |
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(1. 海军军医大学(第二军医大学)东方肝胆外科医院泌尿外科, 上海 201805;
2. 海军军医大学(第二军医大学)东方肝胆外科医院病理科, 上海 201805
△共同第一作者
*通信作者) |
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| 摘要: |
| 目的 总结肾集合管癌的临床诊治经验及进行预后分析。方法 回顾性分析海军军医大学（第二军医大学）长海医院、长征医院和东方肝胆外科医院收治的6 950例肾癌患者资料，将其中经病理确诊为肾集合管癌的21例患者纳入本研究。分析21例肾集合管癌患者的临床资料、病理学资料、影像学资料、手术情况、术后辅助治疗情况及随访资料。结果 肾集合管癌患者占同期肾癌患者的0.3%（21/6 950）。21例肾集合管癌患者中，男18例、女3例，平均年龄为（55±13）岁。症状以血尿、腰痛为主。计算机断层扫描显示病变肾脏体积增大，轮廓欠光滑，肿块病灶边界欠清，增强后不均匀强化。肿瘤最大径为2.4~8.5 cm，平均（5.6±1.7）cm。5例患者伴淋巴结转移，6例伴远处转移。TNM临床分期Ⅰ期8例、Ⅱ期2例、Ⅲ期5例、Ⅳ期6例。20例患者接受手术治疗，1例因全身状态较差未手术。术后病理结果显示肿瘤切面呈灰白或灰黄色，浸润性生长，肿瘤组织排列成不规则腺管状、乳头状，部分呈鞋钉样，间质可见大量纤维组织增生及炎症细胞浸润；免疫组织化学染色显示极低分子细胞角蛋白（CAM5.2）、肿瘤相关上皮膜抗原（EMA）、配对盒基因8（PAX8）、细胞角蛋白7（CK7）表达阳性，碳酸酐酶Ⅸ（CAⅨ）、原癌基因Ⅲ型跨膜酪氨酸激酶受体蛋白（C-kit）、GATA结合蛋白3（GATA-3）、中性内肽酶（CD10）、转化相关蛋白63（P63）、细胞角蛋白20（CK20）表达阴性。16例患者得到随访，平均随访时间为4~87（33.6±28.9）个月，中位生存时间为39.1个月，1年、2年、5年总体生存率分别为71.5%、57.2%、44.5%。12例死亡患者平均生存时间为（32.2±27.5）个月。结论 肾集合管癌是一种罕见的肾细胞癌亚型，病程短，进展快，恶性程度高，预后不良；病理检查是确诊的金标准；手术治疗是目前主要的治疗方法，辅助治疗可以采用化学治疗和靶向治疗；早诊断、早治疗是患者获得良好预后的关键。 |
| 关键词: 肾肿瘤 肾集合管癌 诊断 治疗 预后 |
| DOI：10.16781/j.0258-879x.2019.11.1208 |
| 投稿时间：2019-04-16修订日期：2019-06-26 |
| 基金项目:上海市科学技术委员会青年科技英才扬帆计划（19YF1459700）. |
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| Collecting duct renal cell carcinoma: diagnosis and treatment experiences and prognostic analysis |
| YANG Qi-wei1△,YANG Wei1△,XU Meng-wei2,YE Jian-qing1,GAN Si-shun1,PAN Xiu-wu1,WANG Lei1,TIAN Yi-jun1,CAO Jian-wei1,CUI Xin-gang1* |
(1. Department of Urology, Eastern Hepatobiliary Surgery Hospital, Naval Medical University(Second Military Medical University), Shanghai 201805, China;
2. Department of Pathology, Eastern Hepatobiliary Surgery Hospital, Naval Medical University(Second Military Medical University), Shanghai 201805, China
△Co-first authors.
* Corresponding author) |
| Abstract: |
| Objective To summarize the clinical experiences for diagnosis and treatment of collecting duct renal cell carcinoma (CDRCC) and to analyze its prognosis. Methods A total of 21 CDRCC patients selected from 6 950 patients with renal cell carcinoma admitted to Changhai Hospital, Changzheng Hospital and Eastern Hepatobiliary Surgery Hospital of Naval Military Medical University (Second Military Medical University) were enrolled in this retrospective study. CDRCC was confirmed by pathological examination. Clinical data, pathological data, imaging data, surgical conditions, postoperative adjuvant treatment and follow-up information of the 21 patients were analyzed. Results The proportion of CDRCC patients was 0.3% (21/6 950). There were 18 males and 3 females, with an average age of (55±13) years. The main symptoms were hematuria and flank pain. Computed tomography showed that the size of the kidney involved was enlarged, the outline of the mass was not smooth, and the boundary of the mass was not clear. After enhancement, the mass was heterogeneously enhanced. The maximum diameter of tumor ranged from 2.4 cm to 8.5 cm, with an average of (5.6±1.7) cm. Lymph node metastases were observed in 5 patients and distant metastasis in 6 patients. TNM clinical stage:8 cases in stage Ⅰ, 2 cases in stage Ⅱ, 5 cases in stage Ⅲ and 6 cases in stage Ⅳ. Twenty patients received surgical treatment, but one did not because of poor general condition. The pathological features of the tumors were grey-white or grey-yellow in section, infiltrating growth, irregular glandular tubular and papillary tissues, some of which had hobnail appearance, interstitial fibrous tissue proliferation and inflammatory cell infiltration. Immunohistochemical staining showed that very low molecular cytokeratin (CAM5.2), tumor-associated epithelial membrane antigen (EMA), paired box gene 8 (PAX8), and cytokeratin 7 (CK7) were positive, while carbonic anhydrase Ⅸ (CAⅨ), proto-oncogene tyrosine-protein kinase kit (C-kit), GATA binding protein 3 (GATA3), neutral endopeptidase (CD10), transformation-related protein 63 (P63), and cytokeratin 20 (CK20) were negative. Sixteen patients were followed up for (33.6±28.9) months on average (range, 4 to 87 months). The median survival time was 39.1 months. One-, two-and five-year survival rate was 71.5%, 57.2%, and 44.5%, respectively. The average survival time of 12 dead patients was (32.2±27.5) months. Conclusion CDRCC is a rare subtype of renal cell carcinoma with short course, rapid progression, high degree of malignancy and poor prognosis. Pathological examination is the golden standard for the diagnosis and surgery is the main treatment at present. Chemotherapy and targeted therapy can be used as adjuvant therapy. Early diagnosis and treatment are the key to a favorable prognosis. |
| Key words: kidney neoplasms collecting duct renal cell carcinoma diagnosis treatment prognosis |
