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Carney complex (CNC) is a rare genetic condition in which people typically have pigmented (dark) areas on their skin, as well as a higher risk of certain types of tumors.
In addition to having dark, pigmented areas on their skin, people with CNC are at higher risk for certain tumors including:
Signs of CNC typically appear during childhood or when a person is in their teens or early 20s.
Hyperpigmented (darker) skin spots are the most common sign of CNC. They tend to increase in number during puberty and may fade as people get older.
Cardiac myxomas are also common. While these tumors are non-cancerous (because they don’t spread to other parts of the body), they can still cause serious health issues. These tumors can reduce blood flow in the heart and increase the risk of blood clots (which can lead to a stroke), and they can be life-threatening. Myxomas can also develop on the skin or in the mouth, breast, or other organs.
Endocrine tumors of the adrenal and pituitary glands and of the testicles (in males) are also common in people with CNC. Tumors in these glands or organs can result in abnormally high levels of specific hormones in the body:
Most often, CNC is caused by a mutation in the PRKAR1A gene. More than 130 different PRKAR1A mutations have been found. Most of these mutations have only been identified in a single family.
Changes in other genes are also thought to cause CNC in some people.
Most people with CNC inherit a mutated PRKAR1A gene from a parent. A person with CNC has a 50/50 chance of passing the PRKAR1A gene mutation on to each of their children. However, in about 1 in 4 people with CNC, the gene mutation isn’t inherited from a parent. Instead, the mutation is a random event that happens very early in a person’s life (possibly even before birth). Then it can be passed down to that person’s children.
Options exist for people who carry a PRKAR1A gene mutation and might want to have children. For more information, talk with an assisted reproduction specialist at a fertility clinic.
CNC is rare. Only several hundred cases have ever been reported worldwide.
People are diagnosed with Carney complex based mainly on if they have certain criteria. CNC can be diagnosed:
Major diagnostic criteria for CNC include:
Because Carney complex is so rare, there are no standard screening guidelines for people with CNC. However, recommendations from medical experts to look for tumors early typically include:
Screening recommendations may change over time as new technologies are developed and more is learned about Carney complex. It’s important to talk with your health care team about appropriate screening tests.
Treatment of CNC is different for each person, depending on the type of tumor(s) they have, their symptoms, and other factors.
In general, most types of tumors resulting from Carney complex are removed with surgery if they are causing symptoms (or if they might cause problems in the future). Other types of treatments might also be helpful in some situations. Close follow-up is usually recommended after treatment to watch for possible signs of a tumor recurring (coming back) or new tumors forming.
For some types of tumors that aren’t likely to cause health issues, watching them closely (instead of removing them right away) might be an option.
If you are concerned about your risk of Carney complex or the tumors it can cause, talk with your health care team. It might be helpful to bring someone along to your appointments to take notes. Consider asking your health care team the following questions:
Developed by the American Cancer Society medical and editorial content team with medical review and contribution by the American Society of Clinical Oncology (ASCO).
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Correa R, Salpea P, Stratakis CA. Carney complex: An update. Eur J Endocrinol. 2015;173(4):M85-97.
Cowen EW. Carney complex. UpToDate. 2023. Accessed at https://www.uptodate.com/contents/carney-complex on January 24, 2024.
MedlinePlus [Internet]. Bethesda (MD): National Library of Medicine (US). Carney Complex. 2010. Accessed at https://medlineplus.gov/genetics/condition/carney-complex on January 24, 2024.
Stratakis CA. Carney complex: A familial lentiginosis predisposing to a variety of tumors. Rev Endocr Metab Disord. 2016;17(3):367-371.
Vindhyal MR, Elshimy G, Elhomsy G. StatPearls [Internet]. StatPearls Publishing; Treasure Island (FL): Jul 10, 2023. Carney Complex.
Last Revised: March 1, 2024
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