Mermerkaya Murat, Burgu Berk, Hamidi Nurullah, Yüksel Seher, Özçakar Zeynep B, Sertçelik Ayşe, Yalçinkaya Fatoş, Soygür Tarkan
Departments of *Urology †Pathology ‡Pediatric Nephrology, University of Ankara, School of Medicine, Ankara, Turkey.
J Pediatr Hematol Oncol. 2013 Oct;35(7):e309-10. doi: 10.1097/MPH.0b013e3182707321.
Mayer-Rokitansky-Küster-Hauser anomaly originates from agenesis of the Müllerian duct including agenesis of the uterus and the vagina because of abnormal development of the uterine ducts. This syndrome may be accompanied by the upper urinary tract anomalies such as unilateral renal agenesis, ectopia of 1 or both kidneys, renal hypoplasia, horseshoe kidney, and hydronephrosis. We report a 16-year-old girl, with unilateral renal agenesis, herniating ovary, and renal cell carcinoma in her solitary kidney, associated with Mayer-Rokitansky-Küster-Hauser syndrome-the first case in the literature to our knowledge.
迈耶-罗基坦斯基-库斯特-豪泽综合征源于苗勒管发育不全,包括子宫和阴道发育不全,是由于子宫管发育异常所致。该综合征可能伴有上尿路异常,如单侧肾缺如、一侧或双侧肾脏异位、肾发育不全、马蹄肾和肾积水。我们报告一名16岁女孩,患有单侧肾缺如、卵巢疝,其孤立肾中出现肾细胞癌,与迈耶-罗基坦斯基-库斯特-豪泽综合征相关——据我们所知,这是文献中首例此类病例。