Men born with congenital adrenal hyperplasia (CAH) have an impaired ability to produce the hormones cortisol and aldosterone from their adrenal glands, the glands that are positioned above the kidneys. This results in an overproduction of the signaling hormone adrenocorticotropic hormone (ACTH) from the pituitary gland to try to push the adrenals to make these hormones. This ultimately results in overgrowth of the adrenal glands and overproduction of testosterone from the adrenal glands as a result. This in turn, leads to a decline in the signaling hormones telling the testicles to produce testosterone and sperm. This itself, can lead to subfertility in men with CAH.
Some men with CAH will also experience growth of benign, non-cancerous tumors in the testicles known as testicular adrenal rest tumors (TARTs). They are typically in both testicles when present and can block the tubules (rete testis) allowing sperm to leave the testicles and can worsen infertility by this obstruction. There have been reported cases where other physicians have done sperm retrievals from the testicles of men with TARTs to use with in-vitro fertilization/intracytoplasmic sperm injection (IVF/ICSI). Other cases have been reported where surgeons have removed the testicular tumors and then checked semen analyses to see if the tubules could be unblocked, the men continued to have a sperm count of zero, as scarring from the surgery likely reblocked the outlets or the tissue had already been destroyed by the TARTs not allowing the sperm to have a way to get out. TARTs tend to grow and destroy more testicular tissue as they grow larger.
Dr. Parviz Kavoussi presented his case, which has been the first of its kind described, in a patient in this scenario in which the tumors were removed surgically to minimize further tissue destruction and cure the man’s testicular pain while sperm retrieval was performed at the same time following correction of hormonal parameters. The sperm was frozen and stored to be used in the future with IVF/ICSI to help this man father children when the timing is right.
Dr. Kavoussi comments,” this is the first reported case of its kind to remove the tumors and perform the sperm retrieval at the same surgical setting. This only makes sense, rather than subjecting these men to multiple procedures for the same outcomes. It was wonderful to get to share this with physicians and scientists from all over the world at the American Society of Andrology meeting”.