Patient Education - Endocrine Encyclopedia
Endocrine Surgery Encyclopedia
Definition:
Ectopic Cushing's syndrome is a condition caused by an ACTH-secreting tumor outside
the pituitary or adrenal glands.
Alternative Names: Cushing's syndrome - ectopic
Causes, incidence, and risk factors:
Cushing's syndrome is the result of excess glucocorticoid hormones secreted over
a long period of time. About 15% of Cushing's syndrome cases are due to ectopic Cushing's
syndrome.
Ectopic Cushing's syndrome is caused by tumors that secrete adrenocorticotropic
hormone (ACTH). Other causes of Cushing's syndrome are oversecretion of ACTH by the
pituitary gland, a tumor of the adrenal gland, or long-term administration of corticosteroid
drugs commonly used to treat conditions such as rheumatoid arthritis and asthma.
Small cell tumors of the lung, tumors of the thymus gland, islet cell tumors of
the pancreas, and medullary carcinoma of the thyroid are tumors that can secrete
ACTH, although this occurs rarely.
Symptoms:
Ectopic Cushing's syndrome tends to present with less dramatic features than classic
Cushing's syndrome, but with higher blood pressure and lower potassium. Weight loss
may be present due to underlying cancer.
Classic features of Cushing's syndrome are:
- moon face (round, red, and full)
- "buffalo hump" -- collection of fat between the shoulders
- central obesity -- protruding abdomen and thin extremities
- weakness
- headache
- acne or superficial skin infections
- thirst
- increased urination
- purple striations on the skin of the abdomen, thighs, and breasts
- mental changes
- impotence or cessation of menses (loss of menstrual periods)
Signs and tests:
Tests are done to confirm hypercortisolism, then further tests are done to determine
the cause.
Tests that confirm hypercortisolism:
- a 24-hour urine for cortisol and creatinine
- a dexamethasone suppression test
- serial serum cortisol levels
Test that determines cause:
- serum ACTH, elevated
- Further signs:
- fasting glucose, elevated
- serum potassium, low
Treatment:
The best treatment for ectopic Cushing's syndrome is surgical removal of the tumor.
This is usually possible when the tumor is benign (noncancerous).
However, many tumors are malignant (cancerous), and have metastasized (spread
to other areas of the body) before cortisol excess has been diagnosed,. Surgical
removal is not possible in these situations, and drugs to suppress cortisol secretion
may be given. They include: ketoconazole, aminoglutethimide, and metyrapone.
Support Groups:
Expectations (prognosis):
Surgical removal of the tumor may lead to full recovery, but there is a chance of
the tumor coming back. Survival for people with ectopic tumors depends upon the outcome
associated with the particular tumor type.
Complications:
- the tumor may spread
- excess cortisol may continue
Calling your health care provider:
Call your health care provider if symptoms of Cushing's syndrome appear.
Prevention:
Prompt treatment of tumors may reduce the risk in some cases. Many cases are not
preventable.
Review Date: 1/18/2006
Reviewed By: Nikheel S. Kolatkar, MD, Clinical and Research Fellow, Division of Endocrinology,
Diabetes, and Hypertension, Brigham and Women's Hospital, Harvard Medical School,
Boston, MA. Review provided by VeriMed Healthcare Network.
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