Ectopic Cushing's syndromeSymptoms, Doctors, Treatments, Advances & More
Learn About Ectopic Cushing's syndrome
Ectopic Cushing syndrome is a form of Cushing syndrome in which a tumor outside the pituitary gland produces a hormone called adrenocorticotropic hormone (ACTH).
Cushing syndrome - ectopic; Ectopic ACTH syndrome; Ectopic Cushing's syndrome
Cushing syndrome is a disorder that occurs when your body has a higher than normal level of the hormone cortisol. This hormone is made in the adrenal glands. Too much cortisol can be due to various problems. One of the causes is if there is too much of the hormone ACTH in the blood. ACTH is usually made by the pituitary in small amounts and then signals the adrenal glands to produce cortisol. Sometimes other cells outside the pituitary can make large amounts of ACTH. This is called ectopic Cushing syndrome. Ectopic means that something is occurring in an abnormal place in the body.
Ectopic Cushing syndrome is caused by tumors that release ACTH. Tumors that can, in rare cases, release ACTH include:
- Benign carcinoid tumors of the lung
- Islet cell tumors of the pancreas
- Medullary carcinoma of the thyroid
- Small cell tumors of the lung
- Tumors of the thymus gland
Ectopic Cushing syndrome can cause a lot of different symptoms. Some people have many symptoms while others have only a few. Most people with any type of Cushing syndrome have:
- Round, red, and full face (moon face)
- Slow growth rate in children
- Weight gain with fat accumulation on the trunk, but fat loss from the arms, legs, and buttocks (central obesity)
Skin changes that are often seen:
- Skin infections
- Thick, purple stretch marks [1/2 inch (in) or 1 centimeter (cm) or more wide] called striae, on the skin of the abdomen, thighs, upper arms, and breasts
- Thin skin with easy bruising
Muscle and bone changes include:
- Backache, which occurs with routine activities
- Bone pain or tenderness
- Collection of fat between the shoulders and above the collar bone
- Rib and spine fractures caused by thinning of the bones
- Weak muscles, especially of the hips and shoulders
Body-wide (systemic) problems may include:
- Type 2 diabetes mellitus
- High blood pressure
- High blood cholesterol and triglycerides (hyperlipidemia)
Women may have:
- Excess hair growth on the face, neck, chest, abdomen, and thighs
- Periods that become irregular or stop
Men may have:
- Decreased or no desire for sex
- Impotence
Other symptoms that may occur include:
- Mental changes, such as depression, anxiety, or changes in behavior
- Fatigue
- Frequent infections
- Headache
- Increased thirst and urination
Treatment for ectopic Cushing syndrome is surgery to remove the tumor that is producing too much ACTH. Surgery is usually possible when the tumor is noncancerous (benign).
In some cases, the tumor is cancerous and spreads to other areas of the body before your provider can discover the problem with cortisol production. Surgery may not be possible in these cases. But your provider may prescribe medicines to block cortisol production.
Sometimes removal of both adrenal glands is needed if the tumor cannot be found and medicines do not fully block cortisol production.
Domino's Farms
Dr. Richard Auchus is a professor of Internal Medicine, Division of Metabolism, Endocrinology & Diabetes (MEND) and Chief of the Endocrinology & Metabolism Section at the Ann Arbor VA Medical Center. He received his medical degree and Ph.D. in pharmacology from Washington University in St. Louis. Then Dr. Auchus completed a residency at the University of Iowa and an endocrinology fellowship at UTHSC-San Antonio/Wilford Hall Medical Center. Dr. Auchus came to UMHS from the Graduate School of Biomedical Sciences in Southwestern Medical School, University of Texas, where he was a Professor of Internal Medicine - Endocrinology.Dr. Auchus is a steroid biologist with expertise both in basic science and clinical/translational science. His work has included translational research into: molecular and genetic mechanisms of human hypertension, improved diagnostic studies and management in primary aldosteronism and Cushing syndromes, modifier genes in 21-hydroxylase deficiency, cardiovascular disease in polycystic ovary syndrome, and the endocrinology of traumatic brain injury. Dr. Auchus is rated as an Elite provider by MediFind in the treatment of Ectopic Cushing's syndrome. He is also highly rated in 20 other conditions, according to our data. His clinical expertise encompasses Congenital Adrenal Hyperplasia (CAH), Intersex, Cushing's syndrome, Hormone Replacement Therapy (HRT), and Orchiectomy. Dr. Auchus is board certified in Endocrinology, Diabetes & Metabolism.
Dept Of Transfusion Medicine/Nih Clinical Center
Lynnette Nieman is an Endocrinologist practicing medicine in Bethesda, Maryland. Dr. Nieman is rated as an Elite provider by MediFind in the treatment of Ectopic Cushing's syndrome. She is also highly rated in 8 other conditions, according to our data. Her clinical expertise encompasses Cushing's syndrome, Ectopic Cushing's syndrome, Cushing's disease, and Pituitary Tumor.
Northeast Georgia Physicians Group Endocrinology
Yamuna Gorantla is an Endocrinologist practicing medicine in Gainesville, Georgia. She has been practicing medicine for over 16 years. Dr. Gorantla is rated as an Advanced provider by MediFind in the treatment of Ectopic Cushing's syndrome. She is also highly rated in 33 other conditions, according to our data. Her clinical expertise encompasses Hyperparathyroidism, Thyroid Storm, Hyperthyroidism, and Hashimoto Thyroiditis. Dr. Gorantla is board certified in American Board Of Internal Medicine, Endocrinology Diabetes And Metabolism - 2020 and American Board Of Internal Medicine, Internal Medicine - 2018. Dr. Gorantla is currently accepting new patients.
Surgery to remove the tumor may lead to full recovery. But there is a chance that the tumor will come back.
The tumor may spread or return after surgery. A high cortisol level may continue.
Contact your provider if you develop symptoms of Cushing syndrome.
Prompt treatment of tumors may reduce the risk in some cases. Many cases are not preventable.
Summary: Between 10% and 15% of patients with endogenous hypercortisolism (Cushing syndrome) have ectopic (non-pituitary) production of adrenocorticotropin hormone (ACTH) that causes cortisol excess. In approximately 50% of these patients, the tumoral source of ACTH cannot be found initially despite very detailed and extensive imaging, including studies such as computed tomography, magnetic resonance imagi...
Summary: A Phase 1b/2a, first-in-disease, open-label, multiple-ascending dose exploratory study to evaluate safety, tolerability, pharmacokinetics (PK), and pharmacodynamic biomarker responses associated with CRN04894 (an adrenocorticotropic hormone \[ACTH\] receptor antagonist) in participants with ACTH-dependent Cushing's syndrome (Cushing's disease or Ectopic ACTH Syndrome \[EAS\])
Published Date: April 24, 2025
Published By: Sandeep K. Dhaliwal, MD, board-certified in Diabetes, Endocrinology, and Metabolism, Springfield, VA. Also reviewed by David C. Dugdale, MD, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.
Auchus RJ, Pandey CEF. The adrenal cortex. In: Melmed S, Auchus RJ, Goldfine AB, Rosen CJ, Kopp PA, eds. Williams Textbook of Endocrinology. 15th ed. Philadelphia, PA: Elsevier; 2025:chap 13.
Nieman LK. Adrenal cortex. In: Goldman L, Cooney KA, eds. Goldman-Cecil Medicine. 27th ed. Philadelphia, PA: Elsevier; 2024:chap 208.