Oophorectomy versus salpingo-oophorectomy in Turner syndrome patients with Y-chromosome material: clinical experience and current practice patterns assessment

J Pediatr Surg. 2014 Nov;49(11):1585-8. doi: 10.1016/j.jpedsurg.2014.06.012. Epub 2014 Jul 23.

Abstract

Background/purpose: Gonadectomy is recommended in Turner syndrome (TS) patients with Y-chromosome material due to high risk of tumor in the dysgenetic gonads. No recommendations exist on whether concurrent salpingectomy should be performed.

Methods: A retrospective chart review of surgical procedure and histopathology in TS patients with Y-chromosome enrolled in a TS database was undertaken at Cincinnati Children's Hospital Medical Center. An electronic survey was sent to members of the International Pediatric Endosurgery Group to assess prevalent practice patterns and attitudes on gonadectomy and concurrent salpingectomy in this population.

Results: In March 2011, 12/158 (8%) TS girls (mean age 6.6 years) enrolled in the database had TS with Y-chromosome. Gonadoblastoma was identified in 4/12 (33%) patients and 2/4 had malignant transformation to dysgerminoma and teratoma. Approach to gonadectomy was varied and 3/12 had concurrent salpingectomy. Fifty-four laparoscopic surgeons responded to the survey with no clear consensus on whether salpingectomy should be concurrently performed.

Conclusions: TS patients with Y-chromosome have an increased risk of gonadal tumor development and gonadectomy is recommended. While there is no consensus among pediatric laparoscopic surgeons on concurrent salpingectomy, it is reasonable to consider this combination procedure.

Keywords: Gonadoblastoma; Laparoscopic gonadectomy; Salpingectomy; Turner syndrome; Y-chromosome.

MeSH terms

  • Adolescent
  • Animals
  • Child
  • Child, Preschool
  • Chromosomes, Human, Y
  • Dysgerminoma / surgery
  • Female
  • Gonadoblastoma / surgery
  • Humans
  • Infant
  • Infant, Newborn
  • Laparoscopy / adverse effects
  • Male
  • Noonan Syndrome / genetics
  • Noonan Syndrome / surgery*
  • Ovarian Neoplasms / surgery*
  • Ovariectomy*
  • Retrospective Studies
  • Salpingectomy*
  • Turner Syndrome / genetics
  • Turner Syndrome / surgery*