Han Dong Seok, Yuk Seung Mo, Youn Chang Shik, Park Geon, Sul Hae Joung, Jang Hoon
Department of Urology, College of Medicine, The Catholic University of Korea, Seoul, Republic of Korea.
Department of Radiology, College of Medicine, Catholic University of Korea, Seoul, Republic of Korea.
World J Surg Oncol. 2015 Nov 26;13:324. doi: 10.1186/s12957-015-0739-7.
Primary mucinous adenocarcinoma of the renal pelvis is extremely rare, with only ~100 cases reported till now. Its presumed pathogenesis includes glandular metaplasia of the urothelium of the calyces and the pelvis and malignant transformation of the metaplasia. Unfortunately, it has no characteristic symptoms or radiological features. We report a case of primary mucinous adenocarcinoma of the renal pelvis misdiagnosed as ureteropelvic junction stenosis with a renal pelvis stone.
A 50-year-old man presented with discomfort in his right flank after a fall. A physical examination was normal except mild costovertebral angle tenderness on the right side. The results of most laboratory tests were within normal limits. Plain radiography of the kidneys, ureter, and urinary bladder showed a large radio-opaque mass in the right kidney. Abdominal computed tomography showed a hyperdense mass with 2.62 × 5.70 cm size in the right renal pelvis and severe hydronephrosis and cortical thinning. Diuretic-enhanced 99mTc DTPA renal scanning showed that the relative function of the right versus the left kidney was 20 versus 80 %. On the basis of the imaging findings, kidney dysfunction due to ureteropelvic junction stenosis with a large stone was initially diagnosed. However, the drained urine volume was almost zero, and gelatinous material was aspirated when percutaneous nephrostomy was performed for decompression of hydronephrosis. Although the cytopathology of gelatinous material was negative for malignancy, we could not rule out other disease, such as hidden malignancies of the kidney. We therefore performed radical nephrectomy, and pathological examination of the kidney uncovered a mucinous cystadenocarcinoma in the renal pelvis. A bone scan and positron emission tomography showed no evidence of other malignancies, metastasis, or remnant cancer. The patient has been well, without evidence of tumour recurrence or metastasis, for 20 months after surgery.
Primary mucinous adenocarcinomas of the renal pelvis are extremely rare, and most are diagnosed via post-operative analysis of resected specimens. Although preoperative diagnosis is difficult, urologists should consider the possibility of primary mucinous adenocarcinoma in patients with severe hydronephrosis accompanied by renal stones and chronic inflammation.
肾盂原发性黏液腺癌极为罕见，迄今为止仅报道了约100例。其推测的发病机制包括肾盂和肾盏尿路上皮的腺化生以及化生的恶性转化。不幸的是，它没有特征性症状或影像学特征。我们报告一例肾盂原发性黏液腺癌被误诊为输尿管肾盂连接处狭窄伴肾盂结石的病例。
一名50岁男性在跌倒后出现右侧腰部不适。体格检查除右侧轻度肋脊角压痛外均正常。大多数实验室检查结果在正常范围内。肾脏、输尿管和膀胱的X线平片显示右肾有一个大的不透射线肿块。腹部计算机断层扫描显示右肾盂有一个大小为2.62×5.70 cm的高密度肿块，伴有严重肾积水和皮质变薄。利尿增强99mTc DTPA肾扫描显示右肾与左肾的相对功能分别为20%和80%。根据影像学表现，最初诊断为输尿管肾盂连接处狭窄伴大结石导致的肾功能障碍。然而，经皮肾造瘘术进行肾积水减压时，引流尿量几乎为零，且吸出了胶冻状物质。尽管胶冻状物质的细胞病理学检查未发现恶性肿瘤，但我们不能排除其他疾病，如隐匿性肾恶性肿瘤。因此，我们进行了根治性肾切除术，肾脏病理检查发现肾盂有黏液性囊腺癌。骨扫描和正电子发射断层扫描未发现其他恶性肿瘤、转移或残留癌的证据。患者术后20个月情况良好，无肿瘤复发或转移迹象。
肾盂原发性黏液腺癌极为罕见，大多数通过对切除标本的术后分析来诊断。虽然术前诊断困难，但泌尿外科医生应考虑重度肾积水伴肾结石和慢性炎症患者发生原发性黏液腺癌的可能性。
