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Case report
Persistent Mullerian duct syndrome: rare presentation in an elderly man
  1. Darshil Sunilbhai Shah,
  2. Utsav Shailesh Shah and
  3. Natarajan Kumaresan
  1. Department of Urology, Sri Ramachandra Medical College and Research Institute, Chennai, Tamil Nadu, India
  1. Correspondence to Dr Utsav Shailesh Shah; shah.utsav.dr{at}gmail.com

Abstract

Persistent Mullerian duct syndrome has been described as a disease of internal male pseudohermaphroditism, a rare autosomal recessive disease, characterised by persistent Mullerian derivatives in patients with male pattern 46, XY karyotype and normal pattern virilisation. We present a case of an elderly man, who on evaluation for bilateral undescended testes was found to have a pelvic mass suggestive of malignant transformation of an undescended testis on imaging. On surgical exploration, uterus with multiple fibroids, bilateral fallopian tubes, cervix and bilateral atrophic testes were identified. Interestingly, in this case, imaging (contrastCT and MRI) had missed Mullerian structures due to varied presentation, but exploration and excision of the structures followed by their histopathology revealed uterine leiomyomas and confirmed other Mullerian structures (bilateral fallopian tubes, cervix) with bilateral testes.

  • urology
  • sexual and gender disorders
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Footnotes

  • Contributors DSS and USS were involved primarily in patient care, prepared manuscript with images. NK was operating surgeon and was involved in guiding throughout the course.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Patient consent for publication Obtained.

  • Provenance and peer review Not commissioned; externally peer reviewed.

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