Cushing’s disease is one of the hardest diagnoses to make, requiring multiple blood, urine, and salivary tests; dynamic hormone stimulatory and inhibitory tests; and at times even blood sampling from near the pituitary (inferior petrosal sinus sampling, or IPSS) to determine that a pituitary tumor is the culprit. When the syndrome is suspected, a patient will need workup to determine what’s causing the hormonal excess. It is recommended that the workup and interpretation be done at a medical center with expertise in neuroendocrine tumors, as accurate diagnosis is fundamental and often hard to derive.
Magnetic resonance imaging (MRI) scan produce detailed images of the brain and allow doctors to detect the presence of a tumor. At times a special MRI protocol is needed as some tumors that cause Cushing disease are very small and regular brain MRIs might not pick them up. The test is noninvasive but requires time in a scanner to produce tiny slices of images that are then combined into three-dimensional pictures. Sometimes the patient will need a special contrast agent in advance to increase the visibility of any abnormality found. At times a Cushing tumor is too small to see on a regular brain MRI, and special sequences imaging or even IPSS are at times needed for proper diagnosis and localization.
Cushing’s disease may be able to be treated surgically, by removing the pituitary tumor causing the hormone overproduction. (See Surgery for a Pituitary Tumor.) In cases where surgery is not recommended, pituitary tumors may be treated with medication to suppress ACTH production from the tumor or cortisol production from the adrenals, or to block its effects at the receptor level. At times pituitary radiation is recommended, or even bilateral adrenalectomy. For each patient the treatment is highly individualized after considering all available surgical, medical, and radiation tools as well as clinical trials.
Pituitary tumors are complex lesions that should be treated at major medical centers, by a team experienced in the diagnosis and treatment of tumors affecting the hormones. The Weill Cornell Medicine Brain and Spine Center is fortunate to be home to an internationally recognized surgical team with advanced skills in minimally invasive procedures to remove pituitary tumors. Neurosurgeon Theodore Schwartz, MD, routinely use endonasal (through the nose) approaches to removing pituitary tumors, with no head or facial scarring. (See video of endonasal surgery technique.) He is also a pioneer in transorbital and supraorbital surgical approaches. The team has operated successfully on tumors that other surgeons have deemed impossible to remove. (See video of patient who had a giant tumor removed endonasally.) Dr. Dobri is well versed in all available medications for Cushing's disease, and has taken care of many patients with complex disease that requires combination medical therapy.
Reviewed by: Georgiana Dobri, M.D.
Last reviewed/last updated: September 2023