Persistant Mullerian duct syndrome with intra-abdominal seminoma

Alasmar, Ali; Abu-Qamar, Adnan; Al-saidah, Nizar; Al-Kaabneh, Awad; Al-Qaralleh, Ayman; Alhiari, Ahmad

doi:10.1016/j.eucr.2020.101342

Cited by 4 publications

(1 citation statement)

References 3 publications

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“…PMDS usually presents incidentally in a male presenting with cryptorchid testes or inguinal hernia in childhood or sometimes in adulthood or testicular tumor or abdominal mass in adulthood [ 3 , 8 , 9 ].…”

Section: Discussionmentioning

confidence: 99%

Persistent Müllerian Duct Syndrome: Understanding the Challenges

Chua

Samnakay

2022

Case Reports in Urology

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Persistent Müllerian duct syndrome (PMDS) is a rare autosomal recessive condition defined by the presence of Müllerian duct-derived structures in an otherwise normally masculinized phenotypical and genotypical (46,XY) male. We describe the case of an infant diagnosed with PMDS, managed and followed up for 7 years. The diagnosis of PMDS was made at laparoscopy at 6 months of age for investigation and management of bilateral impalpable testes. A Müllerian structure resembling a uterus with bilateral fallopian tube-like structures was seen in the pelvis, along with bilateral intra-abdominal testes. Gonadal biopsy confirmed normal testicular tissue. The child underwent successful bilateral two-stage Fowler-Stephens orchidopexies. The Müllerian remnant was preserved to maintain testicular vascularity. At the most recent follow-up, the testes are intrascrotal and normal on palpation. There have been no clinical symptoms or concerns with the Müllerian remnant during surveillance with ultrasound and MRI. To date, there are less than 300 cases described in the medical literature, with limited consensus on management. We reflect on challenges the condition poses, including fertility preservation in PMDS, testicular and Müllerian malignancy risk in PMDS, and optimal management and surveillance of PMDS.

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Section: Discussionmentioning

confidence: 99%

Persistent Müllerian Duct Syndrome: Understanding the Challenges

Chua

Samnakay

2022

Case Reports in Urology

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Persistent Mullerian duct syndrome (PMDS): Case report and review of literature

Alanazi

Aldhowayan

Almuhanna

et al. 2022

Urology Case Reports

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Persistan Müllerian Kanal Sendromu: Kriptorşidizmin Nadir Bir Nedeni

BATMAZ¹,

CAYLAN²,

Uçar³

et al. 2022

Akdeniz Medical Journal

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Persistan Müllerian kanal sendromu (PMKS) oldukça nadir görülen ve genellikle insidental olarak tanı konulan erkek cinsel gelişim bozukluğudur. Anti-Müllerian hormon (AMH) veya reseptöründeki bir mutasyon sonucu Müllerian kanal yapılarının regrese olmamasıyla karakterizedir. Çocukluk çağında kriptorşidizmin eşlik ettiği, erişkin dönemde ise buna bağlı olarak infertilite ve artmış testis kanseri ile ilişkili olan PMKS'un erken dönem tanı ve tedavisi oldukça önemlidir. Fenotip olarak erkek karakterde bireyde çoğunlukla kriptorşidizm için yapılan araştırmalar veya cerrahi tedavi sırasında fark edilir. Bilgisayarlı tomografi (BT) veya manyetik rezonans görüntüleme (MRG) tanıya yardımcı olabilir ancak şüpheli olgularda tanısal laparoskopiden faydalınabilir. Çalışmamızda hematospermi ve inguinal ağrı ile kliniğimize başvuran ve laparoskopi sırasında mesane süperiorunda Müllerian kanal yapılarının bulunmasıyla tanı konulan yirmi bir yaşında erkek hasta sunulmuştur.

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Persistant Mullerian duct syndrome with intra-abdominal seminoma

Cited by 4 publications

References 3 publications

Persistent Müllerian Duct Syndrome: Understanding the Challenges

Persistent Müllerian Duct Syndrome: Understanding the Challenges

Persistent Mullerian duct syndrome (PMDS): Case report and review of literature

Persistan Müllerian Kanal Sendromu: Kriptorşidizmin Nadir Bir Nedeni

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