肾集合管癌临床及病理分析

Clinical and pathological analysis of collecting duct carcinoma of the kidney

目的分析肾集合管癌（collecting duct carcinoma，CDC）的临床及病理特点。方法1999年1月至2010年12月收治CDC患者11例，男6例，女5例。年龄22—67岁，平均55岁。主要症状为血尿、腰腹痛。实验室检查无阳性发现。CT检查示肿瘤直径2．1—8．5cm，平均5．6cm。肿瘤位于肾髓质或同时伴有肾皮质、肾盂浸润，边界不清，病变肾脏外形增大，但肾脏轮廓基本存在，增强后呈不均匀轻～中度强化。结果8例行根治性肾切除术，3例行姑息性肾切除术。肿瘤切面呈灰白色，浸润性生长；以腺管乳头状结构为主，部分肿瘤细胞呈靴钉状突向腺腔内，肿瘤间质纤维组织增生明显，有较多淋巴细胞及浆细胞浸润；免疫组化染色检查UEA-1、EMA、PNA、HMW-CK表达阳性，而CD10表达阴性。10例获得随访，随访时间0．3—8．0年，平均2．8年。随访期间死亡7例，平均生存期为12．5个月，2例无瘤生存分别9个月和8年，1例仍在化疗中，1例失访。结论CDC是一种非常少见的。肾癌类型，确诊主要依据病理学检查，肿瘤恶性程度高，进展迅速，预后差。

Objective To analyze the clinical and pathological features of collecting duct carcinoma （CDC） of the kidney. Methods 11 patients with CDC were analyzed, among which 6 were males and 5 were females. Their age ranged from 22 to 67 years old with a mean age of 55. 4 cases were found by routine health examination, 4 cases were presented with gross hematuria and 3 cases had flank or abdomen pain. The CT scan showed an unclear boundary mass in kidney,with tumor sizes from 2.1 to 8.5 em （ mean 5.6 cm）. Only medullary involvement was present on CT in 3 cases, Medullary and eortieal involvement in 5 eases, Medullary and pelvic involvement in 2 cases, and all involvement in 1 case. Infiltrative lesion has expanded kidney but maintains reniform contour. Contrast-enhanced CT scan showed lesion mild to moderate enhancement eompared with surrounding parenehyma. Results Radical nephrectomy was performed in 8 eases and palliative nephreetomy in 3 cases. All patients were finally diagnosed by pathology. Grossly, the tumor is often appears gray or white. In HE staining, tumor showed prominent tubular or tubulaopapillary struetures with desmoplasia and inflammatory reaction. Occasionally,some tumor eells take on a hobnail appearanee. Immunohistochemical examination showed UEA-1 positive in all cases, EMA positive in 9 cases, PNA positive in 8 eases, and HMW-CK positive in 7 eases. Only 2 patients showed positive CD10. 7 pa- tients died within 6 to 47 months （ mean 12.5 months）, 2 survived with tumor free for 9 months and 8 years respectively, one lost of follow-up, and one patient with distant metastasis is receiving postoperative chemotherapy. Conclusions Collecting duct careinoma of the kidney is a rare histological type of renal cell earcinoma, whose final diagnosis depends on histopathological examination. Rapid progression, highly malignant, poor prognosis are the eharacteristics of this disease.