Risk of Malignancy and Need for Surgery in Pediatric Patients with Morris or Y-chromosome Turner Syndrome: A Multicenter Survey - 05/07/15

Doi : 10.1016/j.jpag.2014.09.015

Ciro Esposito, PhD ^1,^⁎ , Maria Escolino, MD ¹, Vincenzo Bagnara, MD ², Felicitas Eckoldt-Wolke, MD ³, Maciej Baglaj, MD ⁴, Amulya Saxena, PhD ⁵, Dariusz Patkowski, PhD ⁴, Felix Schier, PhD ³, Alessandro Settimi, PhD ¹, Helene Martelli, PhD ⁶, Antonio Savanelli, MD ¹
¹ Department of Translational Medical Sciences, University of Naples “Federico II”, Naples, Italy
² Department of Pediatric Surgery, Catania University, Catania, Italy
³ Department of Pediatric Surgery, Medical University of Jena, Jena, Germany
⁴ Department of Pediatric Surgery, Wrocław Medical University, Wrocław, Poland
⁵ Department of Pediatric Surgery, Chelsea and Westminster Children Hospital, London, UK
⁶ Department of Pediatric Surgery, Hôpital de Bicetre, Paris, France

^∗Address correspondence to: Prof. Ciro Esposito, PhD, Department of Traslational Medical Sciences, “Federico II” University of Naples, Via Pansini 5, 80131 Naples, Italy; Phone: +39 081 746 33 77; fax: +39 081 746 33 61

Sous presse. Épreuves corrigées par l'auteur. Disponible en ligne depuis le Sunday 05 July 2015

Abstract

Study 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.

Design

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.

Conclusion

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.

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Key Words : Laparoscopic gonadectomy, Intersex disorders, Tumor, Genitoplasty, Ovarian cryopreservation