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<p>Persistent Mullerian duct syndrome (PMDS) is a rare disorder of sexual differentiation (DSD) in which individuals with a male genotype and phenotype retain Mullerian duct structures such as the uterus, fallopian tubes, and upper vagina. The condition is frequently associated with bilateral undescended testes (UDT) and, in some cases, an inguinal hernia. We present the case of a 30-year-old male diagnosed with PMDS during surgery for a suspected intra-abdominal testicular tumor. The patient, who had been experiencing persistent peri-umbilical pain, was found to have bilateral UDT, a large abdominal mass, and intra-operative discovery of Mullerian duct remnants. Histopathological examination revealed a seminoma in the right UDT and a remnant uterus with fallopian tubes. The patient is receiving adjuvant chemotherapy. PMDS is typically diagnosed during abdominal surgery for UDT or inguinal hernia. The condition’s management, especially concerning the excision of Mullerian duct remnants due to malignancy risk, is essential to preventing potential malignancy. Despite rare fertility in some cases, PMDS is often associated with infertility due to testicular hypoplasia and ejaculatory duct obstruction. This case emphasizes the importance of early recognition and surgical management of PMDS to reduce the risk of malignant transformation.</p>

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A Case of Persistent Mullerian Duct Syndrome Presenting as a Large Pelvic Mass: An Intra-operative Surprise

  • 期刊论文

  • 发表日期：

    2025年10月27日

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  • Vibhu Jain,
  • Santhosh Irrinki,
  • Sudheer Kumar Devana

摘要

Persistent Mullerian duct syndrome (PMDS) is a rare disorder of sexual differentiation (DSD) in which individuals with a male genotype and phenotype retain Mullerian duct structures such as the uterus, fallopian tubes, and upper vagina. The condition is frequently associated with bilateral undescended testes (UDT) and, in some cases, an inguinal hernia. We present the case of a 30-year-old male diagnosed with PMDS during surgery for a suspected intra-abdominal testicular tumor. The patient, who had been experiencing persistent peri-umbilical pain, was found to have bilateral UDT, a large abdominal mass, and intra-operative discovery of Mullerian duct remnants. Histopathological examination revealed a seminoma in the right UDT and a remnant uterus with fallopian tubes. The patient is receiving adjuvant chemotherapy. PMDS is typically diagnosed during abdominal surgery for UDT or inguinal hernia. The condition’s management, especially concerning the excision of Mullerian duct remnants due to malignancy risk, is essential to preventing potential malignancy. Despite rare fertility in some cases, PMDS is often associated with infertility due to testicular hypoplasia and ejaculatory duct obstruction. This case emphasizes the importance of early recognition and surgical management of PMDS to reduce the risk of malignant transformation.