Combined laparoscopic and radical vaginal treatment of primary vaginal leiomyosarcoma in a patient with unicornuate uterus and pelvic kidney

We report a case of primary vaginal leiomyosarcoma occurring in a patient with a unicornuate uterus and pelvic kidney. The patient was treated with radical partial vaginectomy, unilateral groin dissection, total laparoscopic hysterectomy, and left pelvic laparoscopic lymphadenectomy, followed by adjuvant chemotherapy. Twenty-five months after her original procedure, the patient is alive and free of disease. The exact association between müllerian anomalies and primary malignancies of the genitourinary tract is unclear. It is nonetheless important to be aware of the close embryonic association between the genital and urinary tracts when evaluating such patients and choosing the appropriate surgical and therapeutic approach.