PMDS is a congenital condition in which structures such as the uterus: A practical reader guide

In an unusual case, a 26-year-old man who sought treatment for infertility at RG Hospitals in Delhi was found to have a uterus and fallopian-tube-like structures inside his body, revealing a rare congenital condition that had gone undiagnosed until adulthood.

PMDS is a congenital condition in which structures such as the uterus and fallopian tubes remain in a person with typically male chromosomes and male physical development. The condition is generally diagnosed during childhood, often when a child is being evaluated or treated for an undescended testis. Its detection for the first time in adulthood during an infertility evaluation is particularly uncommon. Given the patient’s age, long-standing undescended testes, severe testicular damage and increased cancer risk, doctors removed the Müllerian structures and both testes through laparoscopic surgery. The most unexpected finding emerged after surgery when the removed tissue was examined under a microscope. Both testes were found to be severely atrophied, while the left testis showed Germ Cell Neoplasia In Situ (GCNIS), a pre-cancerous change that can develop into testicular cancer, the report further added.

It is exceptionally rare, with fewer than 300 cases reported in medical literature. Because the patient came to us with infertility, and the underlying condition had remained undiagnosed until adulthood, “This was an extremely unusual case. Because undescended testes carry a higher risk of testicular cancer, “The testes were also severely atrophied, which was an important concern. We therefore had to approach the surgery carefully, taking into account both the unusual anatomy and the long-term health risks,” he added.

The evaluation showed that he had a persistent Müllerian structure along with both testes located inside the abdomen,” Dr Kharbanda said as quoted by ANI.