Hohenfellner M, Schultz-Lampel D, Lampel A, Steinbach F, Cramer B M, Thüroff J W
Department of Urology, Klinikum Barmen Wuppertal, Mainz, Federal Republic of Germany.
J Urol. 1992 Apr;147(4):1098-102. doi: 10.1016/s0022-5347(17)37486-4.
We report on 3 patients with tumor in a horseshoe kidney, 1 of whom had bilateral tumor (renal cell cancer on the right side and urothelial cancer on the left side). Tumors that arise predominantly in the bridge of a horseshoe kidney can mimic the symptoms of an intra-abdominal disease process. Besides routine diagnostic procedures, angiography is essential to plan the surgical approach, which in principle should be organ-sparing. The literature of the embryology of the horseshoe kidney was reviewed for a relationship between the abnormal renal development and the site of tumorigenesis, and for development of a key for the wide variation of blood supply. Recently reported data suggest that the theory of a mechanical fusion is valid only for horseshoe kidneys with a fibrous isthmus but that an abnormal migration of the posterior nephrogenic area causes the majority of horseshoe kidneys in which the isthmus consists of parenchyma. Development of the isthmus through abnormal migration could predispose this location for renal cell cancer and would explain the varying forms of blood supply. Additionally, this hypothesis supports the previously raised assumption that horseshoe kidneys may be the result of teratogenic factors, which also may be responsible for the known increased incidence of related congenital anomalies and of nephroblastoma.
我们报告了3例马蹄肾合并肿瘤的患者，其中1例为双侧肿瘤（右侧为肾细胞癌，左侧为尿路上皮癌）。主要发生在马蹄肾桥部的肿瘤可模拟腹内疾病过程的症状。除常规诊断程序外，血管造影对于规划手术入路至关重要，原则上手术应保留器官。回顾了马蹄肾胚胎学的文献，以探讨异常肾脏发育与肿瘤发生部位之间的关系，以及为广泛变化的血供制定关键因素。最近报道的数据表明，机械融合理论仅适用于具有纤维峡部的马蹄肾，但后肾发生区的异常迁移导致了大多数峡部由实质组成的马蹄肾。通过异常迁移形成峡部可能使该部位易患肾细胞癌，并可解释血供的不同形式。此外，这一假说支持了先前提出的假设，即马蹄肾可能是致畸因素的结果，这也可能是已知相关先天性异常和肾母细胞瘤发病率增加的原因。
