Welcome to...The UCLA Endocrine Surgical Unit
 

Schedule an Appointment
Endocrine Home
About Us
Our Expert Team
Our Services
First Visit for Patients
Patient Education
  Getting started
  High Calcium
  Parathyroid Surgery Success
  Parathyroid Surgery Priorities
  Benefits of parathyroid surgery
  Thyroid and Parathyroid Disease
  Find a Surgeon
  Parathyroid Surgery FAQ
  About endocrine surgery
  History of endocrine surgery
  Outcomes in endocrine surgery
  Health Care Quality and Safety
  Reputable Affiliates
  Diseases we treat
  Endocrine encyclopedia
  Adrenocortical Carcinoma
  Pheochromocytoma
  Thyroid Cancer in Children
  Thyroid Cancer Staging
Scar Gallery
For Referring Physicians
Distance Surgery Program
International Patients
Insurance Questions
Map-Directions-Parking
Contact Us
Patient Experiences
Endocrine Resources
Research Opportunities
Site Map

UCLA Health System


Schedule an Appointment


Patient Education - Endocrine Encyclopedia

Endocrine Surgery Encyclopedia

Ectopic Cushing's syndrome

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.

A.D.A.M. qualityA.D.A.M., Inc. is accredited by URAC, also known as the American Accreditation HealthCare Commission (www.urac.org). URAC's accreditation program is an independent audit to verify that A.D.A.M. follows rigorous standards of quality and accountability. A.D.A.M. is among the first to achieve this important distinction for online health information and services. Learn more about A.D.A.M.'s editorial policy, editorial process and privacy policy. A.D.A.M. is also a founding member of Hi-Ethics and subscribes to the principles of the Health on the Net Foundation (www.hon.ch).

The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed medical professional should be consulted for diagnosis and treatment of any and all medical conditions. Call 911 for all medical emergencies. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. © 1997- 2007 A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.

A.D.A.M. logo


Diseases of the Endocrine System

Tests and studies: