title: Risk of Malignancy and Need for Surgery in Pediatric Patients with Morris or Y-chromosome Turner Syndrome: A Multicenter Survey.
abstract: OBJECTIVE
  The management of intersex patients with Y-chromosome Turner or Morris syndrome remains a challenge. We report our experience with a multicenter European survey.
  
  
  METHODS
  We collected the data on 18 patients (mean age 10.2 years, range 2-17 years) with Morris (10 patients) or Turner (8 patients) syndrome harboring the Y chromosome who were treated in 1 of 6 European centers of pediatric surgery between 1997 and 2013. All patients were evaluated by use of a multidisciplinary diagnostic protocol. All patients received a bilateral gonadectomy via laparoscopy; only 1 center performed ovarian cryopreservation. Seven patients received a concomitant genitoplasty. Operative notes and histology were reviewed for details.
  
  
  RESULTS
  No conversions to laparotomy and no complications were recorded. For the patients receiving only the gonadectomy, the length of hospital stay was 24-48 hours, whereas for the patients receiving an associated genitoplasty, it was 6-10 days. Specimens were negative for tumors in 83.3% of cases, whereas in 3 patients (16.6%), benign abnormalities (Sertoli cell hyperplasia in 1 patient and ovotestis in 2 patients) were recorded. A malignant tumor was not recorded in our series.
  
  
  CONCLUSIONS
  If the risk of malignancy is considered as the main indication for surgery in case of Turner or Morris syndrome, on the basis of our study, this indication should be reevaluated. However, based on the non-negligible rate of benign abnormalities reported in our series (16.6%), the performance of cryopreservation to preserve fertility and the possibility of performing genitoplasty during the same anesthetic procedure represent additional valid indications for surgery.
authors.affiliation: Department of Translational Medical Sciences, University of Naples "Federico II", Naples, Italy. Electronic address: ciroespo@unina.it.
authors.forename: Ciro
authors.initials: C
authors.lastname: Esposito
authors.affiliation: Department of Translational Medical Sciences, University of Naples "Federico II", Naples, Italy.
authors.forename: Maria
authors.initials: M
authors.lastname: Escolino
authors.affiliation: Department of Pediatric Surgery, Catania University, Catania, Italy.
authors.forename: Vincenzo
authors.initials: V
authors.lastname: Bagnara
authors.affiliation: Department of Pediatric Surgery, Medical University of Jena, Jena, Germany.
authors.forename: Felicitas
authors.initials: F
authors.lastname: Eckoldt-Wolke
authors.affiliation: Department of Pediatric Surgery, Wrocław Medical University, Wrocław, Poland.
authors.forename: Maciej
authors.initials: M
authors.lastname: Baglaj
authors.affiliation: Department of Pediatric Surgery, Chelsea and Westminster Children Hospital, London, UK.
authors.forename: Amulya
authors.initials: A
authors.lastname: Saxena
authors.affiliation: Department of Pediatric Surgery, Wrocław Medical University, Wrocław, Poland.
authors.forename: Dariusz
authors.initials: D
authors.lastname: Patkowski
authors.affiliation: Department of Pediatric Surgery, Medical University of Jena, Jena, Germany.
authors.forename: Felix
authors.initials: F
authors.lastname: Schier
authors.affiliation: Department of Translational Medical Sciences, University of Naples "Federico II", Naples, Italy.
authors.forename: Alessandro
authors.initials: A
authors.lastname: Settimi
authors.affiliation: Department of Pediatric Surgery, Hôpital de Bicetre, Paris, France.
authors.forename: Helene
authors.initials: H
authors.lastname: Martelli
authors.affiliation: Department of Translational Medical Sciences, University of Naples "Federico II", Naples, Italy.
authors.forename: Antonio
authors.initials: A
authors.lastname: Savanelli
mesh_terms: D000293:Adolescent; D013734:Androgen-Insensitivity Syndrome / Q000150:complications* / Q000601:surgery; D002648:Child; D002675:Child, Preschool; D002875:Chromosomes; D012734:Disorders of Sex Development / Q000150:complications* / Q000235:genetics / Q000601:surgery; D005060:Europe; D005260:Female; D006066:Gonads / Q000601:surgery*; D006801:Humans; D010535:Laparoscopy / Q000379:methods; D007902:Length of Stay; D008297:Male; D009369:Neoplasms / Q000150:complications* / Q000453:epidemiology; D011183:Postoperative Complications; D012306:Risk; D011795:Surveys and Questionnaires; D063106:Transgender Persons; D014424:Turner Syndrome / Q000150:complications* / Q000601:surgery
keywords: Genitoplasty; Intersex disorders; Laparoscopic gonadectomy; Ovarian cryopreservation; Tumor