Orsola A, Trias I, Raventós C X, Español I, Cecchini L, Orsola I
Department of Urology, Clínica Plató, Barcelona, Spain.
Urology. 2005 Jan;65(1):49-54. doi: 10.1016/j.urology.2004.08.012.
To describe 3 cases of tumors located in the kidney that may relate collecting (Bellini) duct carcinoma (CDC) to urothelial cell carcinoma (UC). We hypothesized that these distinct tumor types may share a common origin. CDC is a subtype of renal cell carcinoma associated with a highly aggressive course, poor prognosis, and limited response to immunotherapy, behaving similarly to UC.
We present 2 cases of CDC and 1 case of UC of the renal papilla. We compared the clinical presentation and survival rate, together with the radiologic, histologic, and immunostaining (including p53) findings, with strong emphasis on the similarities.
One patient with CDC had a previous history of grade 3, Stage Ta bladder UC. The urothelial carcinoma from the kidney papilla (case 3) presented carcinoma in situ of the adjacent urothelium and displayed mixed characteristics with CDC, namely location, positive staining for Ulex europaeus and pyelonephritic changes. p53 staining showed marked positivity in the tumor of patient 2. Disease progression was rapid, with a median survival of 5.6 months (range 5 to 7).
The results of this study suggest that the broad category of renal cell carcinoma includes a spectrum of lesions. In this range of diseases, CDC might be distinct from conventional renal cell carcinoma but share biologic features with UC, with the consequent implications for management. This association between CDC and UC may reflect the common embryologic origin of collecting duct and urothelial cells, since they derive from progressive branching of the mesonephric (wolffian) duct. Furthermore, the differential cytogenetic expression profiles suggest that the molecular events underlying the development of distal nephron and proximal tubule renal cancers are distinct.
描述3例位于肾脏的肿瘤,这些肿瘤可能与集合管(贝氏管)癌(CDC)及尿路上皮细胞癌(UC)相关。我们推测这些不同的肿瘤类型可能有共同的起源。CDC是肾细胞癌的一种亚型,病程高度侵袭性、预后差且对免疫治疗反应有限,其表现与UC相似。
我们呈现了2例CDC和1例肾乳头UC病例。我们比较了临床表现和生存率,以及放射学、组织学和免疫染色(包括p53)结果,重点关注相似之处。
1例CDC患者既往有3级Ta期膀胱UC病史。肾乳头的尿路上皮癌(病例3)呈现相邻尿路上皮的原位癌,并显示出与CDC的混合特征,即位置、欧洲荆豆凝集素阳性染色和肾盂肾炎样改变。p53染色显示患者2的肿瘤有明显阳性。疾病进展迅速,中位生存期为5.6个月(范围5至7个月)。
本研究结果表明,肾细胞癌这一广泛类别包括一系列病变。在这一系列疾病中,CDC可能与传统肾细胞癌不同,但与UC有共同的生物学特征,从而对治疗有相应影响。CDC与UC之间的这种关联可能反映了集合管和尿路上皮细胞共同的胚胎学起源,因为它们源自中肾(沃尔夫管)的渐进性分支。此外,不同的细胞遗传学表达谱表明,远端肾单位和近端肾小管肾癌发生发展的分子事件是不同的。