摘要
目的探讨肾集合管癌（CDC）的临床病理学特点、诊断方法、鉴别诊断及预后。方法回顾性的分析2015年1月至2017年11月收治的3例肾CDC患者的临床资料。3例均为男性。年龄42-73岁，平均57．5岁。病变位于右肾2例，左肾1例。主要临床表现为血尿、腹部肿块及腰腹部疼痛。实验室检查无异常。CT检查示肿瘤直径3．1-5．1cm，平均3．9cm。肿瘤位于肾髓质及肾盂内，呈等低密度或囊实性密度影，肿块向肾外延伸，边界不清，增强后呈不均匀强化。3例均行根治性肾切除术。结果术后病理检查：肿瘤切面灰白色，质硬，呈浸润性生长；镜下见肿瘤具有小管及小管乳头状结构，间质纤维化，部分伴有肉瘤样分化。免疫组化染色检查Vimentin、CK-L、CKpan、P504S弥漫表达阳性，PAX-2、CK7、EMA表达不同程度的阳性，RCC、KSP、CD10、CD117、MOC-31、TFE3均表达阴性。3例均获得随访，随访时问l-15个月，平均6个月。1例因术后出现全身广泛转移行化疗，化疗方案为氟尿嘧啶1500mg，静脉滴注10-12h，每天1次，5d为1个周期，每月1次，共化疗4个周期，化疗后复查肿瘤标志物，癌胚抗原8ng／ml，糖类抗原19-940kU／L，同时出现腹水，腹水检查见癌细胞。l例因肿瘤转移和复发于术后7个月死亡。1例术后未行其他治疗，无瘤生存。结论肾CDC是非常罕见的肾恶性上皮肿瘤，临床症状明显，侵袭力强，预后差，影像学及超声检查仅起到辅助诊断作用，本病诊断和鉴别诊断主要依靠病理检查。
Objective To investigate the clinicopathological features, diagnosis, differential diagnosis and prognosis of renal collecting duct carcinoma （ CDC ）. Methods The clinical data of 3 patients with renal collecting duct carcinoma, during the period from January 2015 to November 2017, were retrospectively analyzed. 3 patients were male with age ranged from 42 to 73 years old, mean of 57.5 years. Two lesions were located in the right kidney and one in the left kidney. Clinical manifestations were hcmaturia, abdominal mass and waist and abdomen pain. No laboratorial abnormality was found. CT examination showed the tumor diameter ranged from 3.1 to 5.1 cm, mean 3.9 cm. The tumors located in the medullary and renal pelvis with low density or mixed density. Those tumors extended to the peripheral of the kidney, which the boundary was unclear. During enhancement CT, the uneven enhancement effect could be observed. Radical nepheroectomy was performed in all patients. Results Postoperative pathological examination showed surface of incision was gray. The texture of tumor was hard. The invasive growth pattern could be noticed. Under the microscope, the tumors had small ducts and papillary structures of tubules with interstitial fibrosis and some sarcomatous differentiation. Immunohistochemical staining showed strong positive expression of vimentin, CK-L, CKpan and P504S, and positive expression of PAX-2, CK7 and EMA in different degrees. RCC, KSP, CD10, CDll7, MOC-31 and TFE3 were all negative. All 3 cases were followed up from 1 to 15 months with an average of 6 months. One case was treated with chemotherapy because of extensive metastases after surgery. Chemotherapy was performed by dissolving 1 500 mg of fluorouracil in 1 000 ml of 5% normal saline and instillation. It was administered once every 10 - 12 hours and once a day for 5 days in one cycle. However, the outcome was poor. 1 patient died of tumor metastasis and recurrence 7 months after surgery. 1 patient had no tumor remaining after surgery. Conclusions CDC is a very rare malignant epithelial neoplasm in kidney. It has obvious clinical symptoms, strong invasive pattern and poor prognosis. Imaging and ultrasonography only play an auxiliary role in diagnosis. CDC ＂s unique histopathology is the main basis of diagnosis and differential diagnosis.
作者
冯润林
叶春伟
易晓佳
王燕
Feng Runlin;Ye Chunwei;Yi Xiaofia;Wang Yan(Department of Pathology,Second Affiliated Hospital of Kunming Medical University,Kunming 650101,China)
出处
《中华泌尿外科杂志》
CAS
CSCD
北大核心
2018年第11期823-826,共4页
Chinese Journal of Urology
关键词
肾肿瘤
集合管癌
免疫组化
病理诊断及预后
Kidney neoplasms
Collecting duct carcinoma
Immunohistochemistry
Pathological diagnosis and prognosis
