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What Is Cushing's Disease?

Cushing’s disease is a challenging condition. It causes physical problems like weight gain, high blood pressure, and diabetes. These conditions are difficult in and of themselves.

But Cushing’s disease is rare, making it all the more complex. Because only 5-10 people in a million are diagnosed annually, even specialists can miss it. For many, it takes years to get an accurate diagnosis. All the while, the disease takes a toll physically, mentally and emotionally.

Even after diagnosis, treatment is not always curative. The pituitary tumors caused by Cushing’s disease can return after surgery.

For all of these reasons, treating Cushing’s disease requires a team of experts with years of diagnostic and treatment experience. And it takes kindness, empathy and a willingness to listen.

That’s what you’ll find when you turn to Penn Medicine’s Pituitary Center. Whether you’re conducting a self-guided search for answers or your doctor referred you to us, you’ll have access to our:

  • Comprehensive diagnostic and treatment options
  • Wealth of surgical experience specific to Cushing’s disease
  • Insights gained from conducting research and training future physicians

How Cushing’s Disease is Diagnosed

The rarity of Cushing’s disease makes it hard to diagnose. In fact, most endocrinologists treat few or no people with Cushing’s disease in their entire careers. After ruling out other conditions, you and your primary care physician or endocrinologist may suspect Cushing’s disease. If so, the first step is to run a series of tests.

There is no single foolproof test for diagnosing Cushing’s disease. However, the most accurate diagnosis comes from a combination of multiple tests conducted over time by an experienced neuroendocrinologist, including:

  • Tests to sample your urinary cortisol
  • Tests to sample your salivary cortisol levels
  • Dexamethasone suppression tests

If Cushing’s disease is advanced, it may only take a few weeks to confirm a diagnosis. However, a subtler case – which is more common – can take several months to diagnose.

Locating the Tumor

After the initial diagnosis, the next step is to locate the tumor in the body. Nearly 85 percent of people with Cushing’s disease have a small benign (noncancerous) tumor in the pituitary gland, and 15 percent have a tumor in the adrenal gland. In rare cases, Cushing’s disease can lead to tumors in multiple organs.

Testing perfected over the years at Penn – including IPSS and CRH testing – can help to find the tumor. IPSS (Inferior Petrosal Sinus Sampling) is used to confirm if the tumor is located in the pituitary gland. This test is performed by an experienced interventional neuroradiologist to see whether the pituitary gland is making excess hormones, such as ACTH (adrenocorticotropic hormone). CRH (Corticotroph Releasing Hormone Test) is a non-invasive alternative that can be appropriate for some patients and is performed at the Penn Dynamic Testing Unit.

The Penn Pituitary Center is proud to offer a specialized protocol of obtaining MRI images to zero in on Cushing’s tumors.

Throughout this process, our team of experienced neuroradiologists and neuroendocrinologists will read your test results, giving you the best chance of finding the source of Cushing’s disease.

Treating Cushing’s Disease

Treatment for Cushing’s disease varies depending on the size, location and complexity of the tumor. You may need a surgical procedure, medication or radiation therapy.

Surgery

Our neurosurgeons have a stellar track record in removing small Cushing’s tumors of the pituitary gland. However, larger pituitary tumors that have extended into surrounding tissues cannot be cured surgically. Once as much of the tumor has been removed as possible, medication or radiation therapy will be offered. On the other hand, tumors on the adrenal gland can be treated through surgery.

Recovery after surgery takes two days in the hospital followed by a short recovery period at home. The main discomfort after surgery is a stuffy nose, which can last for a couple of weeks.

Treatment After Surgery

People who have surgery for Cushing’s disease tumors remain tumor-free about 75 percent of the time, but in some cases, tumors recur within five years of treatment. In other cases, tests after surgery can show that the tumors were non-curable. In this case, you’ll be given additional treatment within two months of the initial surgery.

Treatment options include:

Can Cushing’s Disease Be Cured?

After treatment, Cushing’s disease remains a problematic disease. Even people who are “cured” face a long journey before life truly returns to “normal.” After surgery to remove a Cushing’s tumor, the body’s cortisol levels drop sharply, and it takes several months for the body to return to normal levels of production.

What’s more, prior to surgery, the body may have been changed by months and often years of higher-than-normal cortisol levels, and this is not reversed the moment the tumor is removed. Instead, weakened muscles and excess fatty tissue requires about a year of physical therapy and dietary intervention to reverse.

Unexpected setbacks are common with Cushing’s disease, but your Penn Pituitary Center doctors will remain with you throughout the entire process. We strive to provide the best quality of life and can point you and your family toward the psychological and psychiatric support that you need.

The Future of Cushing’s Disease

While there’s no cure for Cushing’s disease, Penn physicians and researchers are working to develop better diagnostic and treatment options.

We participate in clinical trials of new medications and continue to partner with disease foundations to raise funds and conduct research into new treatments. For instance, promising research is underway to find the genetic markers that drive Cushing’s disease. This could lead to better predictions of how tumors will respond to treatment.

We also pride ourselves on educating other doctors in order to shorten the period between initial symptoms and a diagnosis, which is currently 6 months to 10 years.

Cushing’s Disease Resources

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