Fertility sparing technique during pelvic exenteration for recurrent vaginal rhabdomyosarcoma

Abstract

We present the first case and describe the surgical technique of anterior pelvic exenteration with uterine preservation in a 17-year-old patient with a recurrent vaginal rhabdomyosarcoma.Surgical technique included a skeletonization of uterine pedicles and ligation of superior vesical and vaginal arteries, adapting abdominal radical trachelectomy technique. Cervix was transected to avoid vaginal opening and tumor spillage. Uterus was reimplanted to a vaginal reconstruction, created with a DIEP, and a continent urinary diversion was performed. Pelvic filling was completed with an omental J-flap.Postoperative course was uneventful and the patient was discharged at day 17th. The last days of her stay were dedicated to self-catheterization education and learning the management of Miami pouch. Her menstrual cycle resumed two months after the surgery. Cervix exhibited a normal appearance during clinical examination eight weeks after surgery and postoperative MRI did not show signs of local recurrence. Unfortunately, distant metastatic relapse occurred three months after surgery and the patient died two months later.Fertility preservation at the time of anterior pelvic exenteration is technically feasible in selected young patients. Further cases are needed to assess the reproducibility of this surgical procedure, the reproductive function of the uterus and the rate of uterine recurrences. Keywords: Uterine conservation, Conservative surgery, Genital tract rhabdomyosarcoma