Nelson Syndrome: Causes, Symptoms, and Treatment
Medically reviewed by Harsha Moole, MD, Internal Medicine. Last reviewed: October 23, 2026.
What kind of doctor treats Nelson Syndrome?
An endocrinologist, a doctor who treats hormone problems, usually leads care for Nelson syndrome. A neurosurgeon, a surgeon who operates on the brain and pituitary gland, may remove the tumor. A direct primary care or concierge doctor can be a first stop for an evaluation and can refer you to a specialist when needed.
What is Nelson syndrome?
Nelson syndrome is a rare hormone disorder. It can arise after both adrenal glands are removed in people with Cushing disease. The adrenal glands sit on top of the kidneys. They help keep hormones in balance.
Cushing disease is caused by a tumor or excess growth of the pituitary gland. The pituitary gland sits just below the base of the brain. A type of pituitary tumor called an adenoma is the most common cause. An adenoma is not a cancer. In Nelson syndrome, that pituitary tumor gets bigger after the adrenal glands are removed.
Fewer people are diagnosed with Nelson syndrome now. This is because removing both adrenal glands is no longer routinely recommended for Cushing disease.
Symptoms
Major signs of Nelson syndrome include:
- Darker skin.
- High blood levels of ACTH, a hormone that tells the adrenal glands to make cortisol.
- A growing pituitary tumor.
- Headaches.
- Vision problems, usually in the area you can see.
- Weak nerves that move the eyes.
- Bleeding of the pituitary tumor.
- Low hormone output from the pituitary gland.
Causes and who gets it
In Cushing disease, a non-cancerous pituitary tumor makes too much ACTH. Nelson syndrome develops when this tumor grows after the adrenal glands are removed.
Without adrenal glands, the body makes no cortisol. The pituitary gland keeps making ACTH. In time, the tumors get bigger or new growths form.
It is found more often in females than in males. This is most true for middle-aged females who have Cushing disease. It usually appears one to four years after the surgery. It can arise up to 24 years later.
How doctors diagnose it
Doctors may suspect it when skin gets darker after both adrenal glands are removed. Blood tests check ACTH levels more than once after surgery. A brain MRI scan can also help.
Other non-cancerous pituitary tumors can look like a Nelson syndrome tumor on scans.
Treatment
The first step is surgery to remove the tumor through the nose. Radiation therapy might be used after surgery.
For some people, frequent scans to watch the tumor may be advised instead of surgery.
People with Nelson syndrome cannot make cortisol and aldosterone. They need hormone supplements. Talk with your doctor about how to change the dose when you are sick. Recent studies show mixed results with drug therapies.
When to see a doctor
Tell your doctor if your skin gets darker after adrenal gland surgery. Also tell your doctor about headaches or vision problems.
After a pituitary tumor is removed, call your provider if you have signs it has returned.
Call 911 or go to the emergency room for fainting, a sudden change in mental state, a sudden change in vision, or sudden, severe pain anywhere in the body, such as a sudden bad headache. Also get emergency care right away if you have a severe infection or diarrhea, or you are vomiting and cannot keep your hormone pills down. These can lead to an adrenal crisis.
How a direct primary care or concierge doctor fits in
A direct primary care doctor charges a flat monthly fee instead of billing insurance, while a concierge doctor usually charges more and often works alongside insurance. A direct primary care doctor can review your history, order first blood tests, and help you keep up with follow-up visits. They can also coordinate care with an endocrinologist and a pituitary surgeon.
Common questions
What causes Nelson syndrome?
The cause is growth of the pituitary tumor after both adrenal glands are removed. The pituitary gland keeps making ACTH.
Is Nelson syndrome common?
No, it is rare. Fewer people get it now because removing both adrenal glands is no longer routinely advised for Cushing disease.
How is Nelson syndrome treated?
Treatment usually starts with surgery to remove the pituitary tumor. Radiation therapy might be used after surgery. People also need hormone supplements.
How long after surgery can Nelson syndrome appear?
It usually appears one to four years after both adrenal glands are removed. It can also arise up to 24 years after the surgery.
Sources
- NORD - Nelson Syndrome
- MedlinePlus - Cushing disease
- National Institute of Diabetes and Digestive and Kidney Diseases - Treatment for Adrenal Insufficiency
Medical Disclaimer
This content is for general educational and informational purposes only and is not medical advice. It is not a substitute for professional medical advice, diagnosis, or treatment, and reading it does not create a doctor-patient relationship. Always seek the advice of your physician or another qualified health provider with any questions about a medical condition, and never disregard or delay seeking professional advice because of something you read here. If you think you may have a medical emergency, call 911 (or your local emergency number) immediately.
