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Bilateral Synchronous Testicular Germ Cell Neoplasia in a 9-year old with Persisitent Mullerian Duct Syndrome (Abstract)

K. Jarvi¹, I Scheimberg²,M Al-Adnani², M O Savage³, CL Cord-Udy¹

¹Paediatric Surgery Department

²Paediatric Pathology Department

³Paediatric Endocrinology Department

Royal London Hospital

 

Abstract

Persistent Müllerian duct syndrome (PMDS) is a rare disorder of male sexual differentiation caused by defective action or synthesis of anti-Müllerian hormone (AMH). Organogenesis of the male external genitalia is not affected. There is failure of regression of Müllerian structures, resulting in the presence of a uterus, fallopian tubes and upper third of the vagina in males with a 46XY karyotype. Uni- or bilateral cryptorchidism may be a presenting feature, and PMDS is not usually diagnosed until surgery for this. Herein the authors report a 9 year old boy with PMDS who was found to have bilateral synchronous testicular germ cell neoplasia on histology following elective orchiectomy and excision of Müllerian remnants. He is the sixth case of bilateral synchronous testicular malignancy in PMDS.

 

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