Prostatic adenocarcinoma in the setting of persistent müllerian duct syndrome: a case report

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Abstract

Persistent müllerian duct syndrome (PMDS) is a form of disordered sex development in which rudimentary müllerian structures are identified in phenotypically and genotypically normal males. It is caused by defects in the anti-müllerian hormone (AMH) system. Since patients with PMDS present with undescended testes, testosterone production by Leydig cells later in life is often decreased. The role of androgens in prostate cancerogenesis is well known. Cryptorchid testes and diminished testosterone levels in post-pubertal life in patients with PMDS play a protective role against prostate cancer, and hence, prostate cancer is a rare event in patients with PMDS. Herein, we present a patient who underwent prostatectomy for high-grade prostatic adenocarcinoma with persistent müllerian structures (such as rudimentary uterus, fallopian tubes, and cervix) identified during surgery. To our knowledge, this is the second case reported in the English language literature where PMDS was associated with prostate cancer.

Original languageEnglish (US)
Pages (from-to)125-131
Number of pages7
JournalHuman Pathology
Volume75
Early online dateNov 11 2017
DOIs
StatePublished - May 1 2018

Keywords

  • Cryptorchidism
  • Persistent müllerian duct syndrome
  • Prostate cancer
  • Rudimentary uterus

ASJC Scopus subject areas

  • Pathology and Forensic Medicine

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