Abstract

Herlyn-Werner-Wunderlich syndrome, a rare Mullerian duct anomaly, includes a triad of uterine didelphys, obstructed haemivagina and ipsilateral renal agenesis. A 58-year-old woman with Herlyn-Werner-Wunderlich syndrome, reported of recurrent genital bleeding for 9 years, was finally diagnosed with endometrial cancer. She had a history of vaginal septum resection and nephrectomy of atrophic right kidney. MRI demonstrated uterine didelphys, a tumour filling the left uterus and a cyst on the right lateral side of the uterus. Robot-assisted hysterectomy, including bilateral salpingo-oophorectomy and pelvic lymphadenectomy, was performed. As the cyst communicated with the right cervix, but not with the urinary tract, a Gartner duct cyst was diagnosed. Uncertain diagnosis and delay of treatment in endometrial cancer may occur in patients with Herlyn-Werner-Wunderlich syndrome. We should preoperatively fully evaluate the anatomy of the uterus and surrounding tissues and plan surgical procedures, especially in patients with urogenital malformations.