Testicular adrenal rest tumors (TARTs) are common in males diagnosed with classic congenital adrenal hyperplasia (CAH). It’s unknown exactly how many people develop these tumors. The Journal of Clinical Research in Pediatric Endocrinology estimates that about 40 percent of male adults living with classic CAH have TARTs, but the number varies from study to study.

Although TARTs are benign (not cancerous), they can lead to long-term testicular damage and infertility. It is important to be able to recognize the symptoms of TARTs so you can seek treatment if necessary.

Here are the facts you need to know about TARTs.

Symptoms of TARTs

TARTs are masses that can develop in the testes. Some people who develop TARTs have pain in their testicles and can see swelling, feel a lump, or both. However, TARTs can usually only be felt by touch when they’re larger than 2 centimeters. By the time they get this big, they may have caused significant damage. Other people won’t have any symptoms at all.

As many as 40 percent of males with CAH will eventually develop a testicular rest tumor.

TARTs are usually found during childhood or the adolescent years, and they may become more common as males with classic CAH become adults. They can continue to grow, causing more damage as they become larger. While they aren’t cancerous and won’t become life-threatening, they can make it harder for the body to produce sperm and maintain the right balance of hormones. Problems with either of these functions can lead to infertility.

Monitoring for TARTs

Due to the risk of infertility, many endocrinology doctors recommend yearly testicular ultrasounds for males living with CAH, even during childhood. This can help find the condition early, so you or your child can get the right treatment sooner and lower the risk of long-term damage. Some doctors may also order an MRI to look for TARTs, although these aren’t necessarily better than ultrasounds.

Causes of Testicular Tumors With CAH

TARTs are most common in males with CAH. However, a few other health conditions can raise the risk for them, too. No one knows exactly what causes TARTs in people with CAH, and researchers are still investigating. Below is a summary of what researchers have learned so far.

Adrenocorticotropic hormone (ACTH) is a hormone made and released by the pituitary gland. ACTH tells your adrenal glands to release cortisol. Many people who live with classic CAH don’t produce enough cortisol or aldosterone. When there’s not enough cortisol, the pituitary gland notices the problem and releases additional ACTH. High levels of ACTH seem to be connected to developing TARTs.

Testicular tumors associated with CAH aren’t cancerous, but they can cause damage and problems with fertility.

There’s some evidence that high ACTH levels aren’t the only cause of TARTs. For instance, even people with well-controlled CAH are more likely to develop TARTs than people without CAH. Additionally, while these tumors are much less common in people with nonclassic CAH, they may show up there, too. This all leads researchers to suspect that high levels of ACTH may not be the only factor that leads to TARTs. Researchers need to do more work to figure out what else might lead to these tumors.

It’s important to note that even if there are other factors that affect the development of TARTs, ACTH is likely to be the most important one. Controlling it may be key to treating TARTs or even eliminating the development of these tumors.

Certain mutations (genetic changes) associated with CAH may make people more likely to develop TARTs. If you know what genetic variant you have that’s associated with your CAH, you can talk to your doctor about whether it increases your risk for TARTs.

Biological Origin of TARTs

Researchers are also uncertain about where the cells come from that form these tumors. TART cells seem to share some characteristics with adrenal cells, so one theory is that they migrated from the adrenal glands before birth, while the baby was still developing. Another theory is that TARTs originate from the same cells that the adrenal glands came from, which is why they respond to ACTH like the adrenal gland.

Treating Testicular Tumors With CAH

To get the right treatment for TARTs, you’ll first need to get the correct diagnosis. Distinguishing between TARTs and cancerous testicular tumors called Leydig cell tumors can be difficult, as the symptoms can appear similar. In general, benign TARTs are more likely to occur on both testicles than cancerous tumors. TARTs also tend to develop in a specific location in the testicle, while cancer can grow in many areas.

There’s no single way to treat TARTs, but most CAH specialists agree that improving hormone control, sometimes by adjusting glucocorticoid doses, may help.

TART cells will look different under a microscope, too. In rare cases, if your healthcare provider isn’t sure what kind of tumor you have, they may order a biopsy so they can make the correct diagnosis and get you or your child the best treatment.

The Most Common Treatment for TARTs

There’s no single way to treat TARTs. Most specialists agree that lowering your ACTH levels may help. To try to do this, doctors may prescribe higher doses of glucocorticoids. This approach may help, but the side effects can be serious.

Taking too many glucocorticoids can be hard on your body. High doses of this medication can lead to weight gain, hypertension (high blood pressure), and stretch marks. In children, it can lead to less overall growth. It’s important to balance the benefits of controlling ACTH levels against the potential risks of these side effects.

Surgery and TARTs

While removing a tumor surgically may be an option, it’s not usually the first one most healthcare professionals recommend for TARTs. In fact, it’s only recommended if you’re in severe pain and other methods of treating the tumor don’t work. Unfortunately, surgery won’t restore lost fertility. This is why it’s vital to find TARTs and treat them as early as possible.

If your doctors mistake your tumors for cancer, they may recommend surgery right away. You’ll want to make sure you have the right diagnosis before you agree to the procedure. Make sure all doctors involved know that you’re living with CAH and that TARTs are a possible complication.

Restoring Fertility

If your fertility is reduced due to TARTs, your endocrinologist may recommend medications to help. However, these may come with significant side effects. You and your doctor will need to weigh the benefits of preserving fertility against the problems that these could cause for you later.

Hormonal treatments may also be available to help you regain fertility. These don’t work for everyone and can have their own side effects. Once again, you and your doctor will need to decide what option is best based on your specific case of CAH and your needs.

Talk to a Doctor

If you or your child lives with CAH, watch for symptoms of testicular masses or tumors. If you notice any lumps or testicular pain, talk to your doctor right away. Finding TARTs early and starting treatment promptly can help protect testicular health and lower the risk of infertility later.

You may also want to ask your doctor whether you or your child should have regular or follow-up ultrasound screenings. For children with CAH, screening may start in childhood. Ultrasounds can help find TARTs early, even before symptoms appear.

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