Objectives Embryonal rhabdomyosarcoma is treated using a multi-modal approach, which can include systemic chemotherapy, radiation, and surgery. When arising from the genital tract, the disease has a 5-year overall survival greater than 80%. However, many of these therapies can result in infertility, which is assured if hysterectomy is performed. Our objective is to describe within the pediatric population an alternative method of obtaining local control surgically through the fertility-sparing approach of an abdominal trachelectomy.
Methods Due to the very narrow vaginal anatomy in pediatric patients, a vaginal trachelectomy approach was not possible, and an abdominal approach was performed. The resection included the cervix and upper vagina. A near-infrared camera was used at the end of the procedure to confirm vascular perfusion to the uterus.
Results A 4-year-old female with a vaginal embryonal rhabdomyosarcoma previously treated with chemotherapy and intravaginal brachytherapy, presented with recurrence on MRI 1.5 years after completing treatment. She underwent a vaginoscopy that demonstrated a pedunculated mass arising from the proximal vagina. A biopsy confirmed recurrence of the primary tumor. Although nearly all visible tumor was resected, positive surgical margins required further surgical resection of a portion of the vaginal wall for local control and further systemic chemotherapy. She underwent an abdominal trachelectomy with preservation of the entire uterus, the majority of the vagina, and negative surgical margins were obtained. She recovered well and is currently undergoing chemotherapy.
Conclusions To our knowledge, this is the youngest patient having undergone an abdominal trachelectomy. This approach appears to be safe in the pediatric population.
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