What Is Carney Complex?
Medically reviewed by Steffini Stalos, DOMedically reviewed by Steffini Stalos, DOCarney complex is an extremely rare condition that causes benign (noncancerous) tumors, called myxomas, and changes to skin pigmentation. Carney complex can be deadly when heart myxomas appear, impacting blood flow and heart function. That can lead to heart attack and myxomatous symptoms, in which the heart valves don’t close completely.However, with proper treatment and monitoring, people with Carney complex can have a normal life expectancy.
Carney Complex vs. Carney TriadCarney complex and Carney triad are two entirely separate conditions. Both are extremely rare and have to do with tumors, which can be confusing, especially since both are sometimes called Carney syndrome. Carney complex is usually diagnosed in teens or adults. Carney triad is diagnosed in children when they have three specific kinds of endocrine tumors that are completely unrelated to Carney complex.
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Who Gets Carney Complex?Carney complex is extremely rare. Fewer than 750 people with the disease have ever been identified. Most of these people have a family history of the condition, although some people have Carney complex even when no one else in their family has it.Most people with Carney complex have a mutation in the PRKAR1A gene. This can be passed from parents to offspring or can occur randomly. Some people with Carney complex do not have this genetic mutation.Researchers are not entirely sure what causes Carney complex in these people, but they believe another type of genetic mutation, likely on the second chromosome, is to blame. Most people are diagnosed as teens or young adults.Start of Symptoms and Tumor ProgressionThe symptoms of Carney complex vary widely. So does the progression of the disease. Some symptoms are present from birth and often become more noticeable during the teen years. People might not have all of the symptoms.Discolored skin and pigmentation changes are often one of the first signs of Carney complex. There are a few different types of skin discoloration associated with the condition, including:Blue nevi are small, raised blue or blue-black spots on the skin. Café au lait spots: Light-brown or brown areas of discoloration, darker than the surrounding skin. They often have irregular borders. Depigmented lesions: Areas of skin that are lighter than the surrounding skin. Lentigines: Small, dark, flat patches that look like freckles are often one of the first signs of Carney complex. They’re most common around the eyes, ears, lower lip, and genitals.The other major symptom of Carney complex is the development of myxomas. These are benign tumors, but they can still be very dangerous since they can cause heart disease. Cardiac myxomas that form in the heart can lead to sudden death, stroke, or symptoms of heart failure.While cardiac myxomas are most common, people with Carney complex can develop myxomas and other types of tumors anywhere, including around nerves. Tumors are especially common in hormone-producing areas of the body, including the:Adrenal glandOvariesPituitary glandTestesThyroidSpecialist DiagnosisA diagnosis of Carney complex is made after a thorough workup. Usually, you’ll see multiple specialists, including a cardiologist (heart specialist), a geneticist (genetics specialist), and an endocrinologist (hormones specialist).There’s not one specific test to diagnose Carney complex. However, your healthcare provider will likely do genetic testing to look for a mutation of the PRKAR1A gene and to rule out other symptoms. In general, if you have two or more of the following symptoms or conditions, healthcare providers will consider Carney complex:Primary pigmented nodular adrenocortical disease (PPNAD)Testicular tumorsAcromegaly (rapid growth caused by the pituitary gland)Cardiac myxomaLentiginosisMelanotic schwannoma, a type of tumor that wraps around nervesMultiple blue neviOsteochondromyxoma, a very rare bone tumorSkin myxomaThyroid tumors
Primary Pigmented Nodular Adrenocortical Disease (PPNAD)PPNAD is a condition that causes the adrenal gland to produce too much cortisol. That can lead to the development of Cushing syndrome. PPNAD can occur on its own, or be triggered by Carney complex. If you have PPNAD and another of the symptoms above, talk with your healthcare provider about the likelihood that you have Carney complex.
Treatment Effect on Life ExpectancyThe life expectancy for someone with Carney complex is 50–55 years. However, most people with Carney complex die from complications from their tumors. With regular management of the tumors, people with Carney complex can have a typical life expectancy.There’s no set treatment for Carney complex. Instead, treatments focus on alleviating symptoms. For example, if you have a tumor in your heart, you may need open heart surgery to remove that tumor and reduce your risk of heart attack or stroke. Possible ComplicationsThe most serious complications from Carney complex are cardiac events, including sudden death or stroke. It’s important for people with Carney complex to work closely with a cardiologist. Tumors can cause other symptoms too, depending on where they appear. For example, tumors on the pituitary gland can cause excessive growth. People who have tumors on the adrenal gland can produce too much cortisol, which in turn can lead to Cushing's syndrome. Surgery is a common treatment for removing tumors caused by Carney complex.
Tumor RecurrenceOftentimes, people with Carney complex experience regrowth of their tumors. Talk with your healthcare provider about the chance of your tumor coming back if it’s removed, and ask if there are any treatments that can lower the risk of tumor recurrence.
Ongoing Monitoring and Follow-Up TestingIf you have Carney complex you’ll work very closely with your healthcare team. You should undergo an ultrasound at least once a year to look for cardiac myxomas. In addition, consider consulting a genetic counselor about testing for you and your family.SummaryCarney complex is an extremely rare disease. Fewer than 750 people have ever been diagnosed with it. The condition is caused by a genetic mutation that leads to skin discoloration and tumor growth throughout the body. The most serious impact of this is cardiac disease, since tumors can grow in the heart. However, with careful monitoring and proactive treatment, people with Carney complex can have a normal life expectancy. Read the original article on Verywell Health.
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