Diseases associated with a high cortisol level, or hypercortisolism, typically come to the attention of patients and their doctors because of the distinctive signs and symptoms associated with excess cortisol. Patients with hypercortisolism commonly experience fatigue, weakness, irritability and an accumulation of abdominal fat. High cortisol levels occur with tumor-related, or neoplastic, diseases that may involve the brain, adrenal glands or other organs. Diagnosis commonly involves laboratory tests and imaging studies to determine the location of the tumor and an appropriate treatment strategy.
Pituitary Adenoma
The pituitary gland is a small endocrine organ, located on the underside of the brain at approximately eye level. The pituitary gland secretes a variety of hormones that stimulate other endocrine glands throughout the body. The front region of the pituitary gland secretes adrenocorticotropic hormone, or ACTH, which stimulates the adrenal glands. A tumor of the pituitary gland, known as a pituitary adenoma, may secrete excessive levels of ACTH. In response to the elevated ACTH level, the adrenal glands produce excessive amounts of hormones, including cortisol. Overproduction of cortisol caused by an ACTH-secreting pituitary adenoma is known as Cushing's disease.
The UCLA Health System Division of Neurosurgery reports that pituitary adenomas occur in approximately one out of every 1,000 adults. Although most pituitary adenomas prove noncancerous, these tumors can invade or compress nearby brain structures. Compression of the nearby eye nerves may threaten vision.
Adrenal Adenoma and Adrenocortical Carcinoma
Some noncancerous and cancerous adrenal gland tumors produce excess cortisol. Adrenal adenomas are noncancerous, or benign, adrenal tumors. Adrenocortical carcinoma is a cancerous tumor of the outer area of the adrenal gland known as the adrenal cortex.
Surgical removal of the adrenal gland, or adrenalectomy, remains the treatment of choice for cortisol-producing tumors, note Drs. Damian Morris and Ashley Grossman on the medical reference website Endotext.com. For patients with a contraindication to surgery or advanced adrenocortical carcinoma, doctors often recommend medical therapy to suppress cortisol production. Medications commonly used to reduce cortisol production include metyrapone, mitotane and ketoconazole.
Ectopic ACTH Syndrome
Certain types of nonpituitary tumors may produce adrenocorticotropic hormone, leading to overstimulation of the adrenal glands and a high cortisol level. This condition, known as ectopic ACTH syndrome, most commonly occurs with specific types of lung tumors, reports the National Institute of Diabetes and Digestive and Kidney Diseases. Tumors of the thyroid, pancreas and thymus gland may also produce ACTH, leading to a high cortisol level.
Surgical removal of the tumor is the preferred treatment option for ectopic ACTH syndrome, according to Morris and Grossman. For patients whose tumors prove cancerous, however, doctors may recommend other treatment options.
