SURGICAL HYSTEROPROSTATECTOMY? A 59-YEAR-OLD MALE PATIENT WITH PROSTATE CANCER FOUND TO HAVE PERSISTENT MULLERIAN DUCT SYNDROME WITH UTERUS AND FALLOPIAN TUBES

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Background: Persistent Mullerian duct syndrome (PMDS) is a rare condition where the Mullerian structures fail to regress completely, resulting in male patients having both male and female internal organs. It most commonly presents in childhood with bilateral intra-abdominal undescended testicles or a unilateral undescended testicle and contralateral inguinal hernia. Here, we present a case of an adult PMDS patient with prostate cancer and a history of bilateral undescended testicles.

Case: A 59-year-old African American patient with past medical history of intravenous drug use (IVDU), congestive heart failure, hepatitis C, infertility, and bilateral cryptorchidism presented to clinic with an elevated prostate specific antigen (PSA). Past surgical history revealed a failed orchidopexy at 11 years old, neither testicle was identified intraoperatively. Prostate biopsy showed unfavorable intermediate risk prostate cancer and the CT scan revealed suspected uterine tissue. Routine prostatectomy with staging was conducted along with additional hysterectomy and bilateral salpingectomy which was sent to pathology. The left testicular vessels were blind ended, while the right had atrophic vessels entering the internal inguinal ring. Pathology revealed positive lymph nodes, which the patient desired to treat with inguinal orchiectomy over androgen suppressive therapy. Orchiectomy revealed an atrophic right testicular nubbin, and the patient’s total free testosterone dropped from 293 ng/dL to 10 ng/dL with removal. To date, he remains asymptomatic with a PSA < 0.1.

Discussion: PMDS is a rare diagnosis, particularly in an older adult. The female structures develop in close proximity to ejaculatory ducts, obstructing them, which is thought to be the main reasons for infertility in PMDS patients. Another theory is azoospermia due to the hostile environment within the abdomen or inguinal canal for spermatogenesis. The Mullerian structures have a low chance of malignant degeneration, however, surgical removal risks damaging surrounding structures, therefore, it is suggested to leave the Mullerian structures. Although our patient never sought treatment for his associated infertility, he had numerous healthcare interactions, no testes on physical exam, a tender inguinal mass, and history of an unsuccessful orchiopexy at age 11 and yet, to our knowledge, never obtained a urology referral. This is particularly concerning as PMDS patients carry a 33% risk of testicular cancer, making early detection critical. This delay could be related to many factors, including the limited access to quality care experienced by IVDUs and the poorer health outcomes experienced by African Americans when compared to Caucasians.

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