What Is Carney Complex?

Carney complex is a rare genetic disorder (multiple endocrine neoplasia syndrome) characterized by an increased risk of multiple benign tumors (familial multiple neoplasias).

The condition is associated with skin pigment abnormalities, myxomas, which are benign or noncancerous connective tissue tumors, and several other tumors of the endocrine (hormone-producing) glands, such as the adrenal gland. The specific symptoms and severity of Carney complex can vary significantly from one person to another.

At Urology of Greater Atlanta, our surgeons offer various treatments for adrenal cancer, depending on your condition’s severity and overall health needs. Your treatment plan could include medication, radiation, or chemotherapy. In some cases, surgery may be necessary to remove the tumor or your adrenal glands.

Book an appointment today with one of our board-certified urologists at any one of our multiple locations throughout Georgia. We look forward to giving you the assistance you need!

What Causes Carney Complex?

Mutations of the PRKAR1A gene are responsible for most cases of Carney complex. These mutations may occur with no family history or be inherited as an autosomal dominant trait.

The PRKAR1A gene is understood to be a tumor suppressor gene. It produces a protein called protein kinase A (PKA) which can suppress or stimulate cell growth and proliferation. A mutation of the PRKAR1A gene leads to increased PKA signaling in affected tissues, leading to various symptoms.

An affected parent will have a 50% chance of passing the abnormal gene to their offspring. Therefore, genetic testing, along with genetic counseling, is recommended for patients with this disorder.

Carney Complex Symptoms

The symptoms of Carney complex can vary greatly from one person to another, even among members of the same family. The disorder may be evident at birth, but the median age of diagnosis is 20.

Many of the signs and symptoms of Carney complex become apparent during the teenage years or early adulthood. The following is a list of the most common symptoms of Carney complex:

• Multiple lentigines — Spotty skin pigmentation usually found around the upper and lower lips, eyelids, lining of eyes, and inside eyelids.
• Blue nevi — Small blue or bluish-black spots on the skin.
• Myxomas — Small benign tumors within the connective tissue. Most commonly found in the heart, but can be found almost anywhere in the body except the hands and feet. Cardiac myxomas can be very dangerous. If one of the valvular openings of the heart becomes blocked, it could cause sudden death.
• Cutaneous myxomas — These are detected as white, pink, or flesh-colored papules or small nodules beneath the skin. They can be found on the eyelids, nipples, external ear canal, or just about anywhere on the body except the hands or feet.
• Endocrine tumors — Tumors can be found within the endocrine system causing endocrine overactivity. The most common tumor associated with Carney complex is a primary pigmented nodular adrenocortical disease (PPNAD). The nodules affect the adrenal glands, causing them to produce higher levels of cortisol. This effect is also known as Cushing syndrome.
• Pituitary adenoma — A benign tumor affecting the pituitary gland. Sometimes results in excessive production of growth hormone resulting in an enlargement of the hands, feet, jaw, and face.
• Thyroid tumors — The tumors are usually benign and do not produce extra hormones. However, those with a history of multiple thyroid nodules may have papillary or follicular thyroid carcinoma.
• Testicular tumors — Affects a third of males with Carney complex. The most common are Leydig cell tumors and pigmented nodular adrenocortical rest tumors. These tumors can sometimes affect fertility.
• Ovarian cysts — Although they have been detected in some women, they are not frequently found.
• Tumor of the peripheral nerve sheath — A very rare tumor that may occur in 10% of patients. They can be found anywhere along the central and peripheral nervous system, gastrointestinal tract, or nerves along the spine.

It is important to note that affected individuals may not have all the symptoms discussed above. Therefore, affected individuals or parents of affected children should talk to their physician and medical team about their specific case, associated symptoms, and overall prognosis.

How Is Carney Complex Diagnosed?

Carney Complex is diagnosed when you have two or more symptoms related to the disorder. Only one symptom is needed to confirm your diagnosis if you already have a close relative that has a positive diagnosis of Carney Complex. Genetic testing can provide a more accurate diagnosis.

How Is Carney Complex Treated?

If you have adrenal cancer due to Carney complex, then our team of experts at Urology of Greater Atlanta can offer you a variety of treatments. Your treatment plan could include medication, radiation, or chemotherapy. In some cases, surgery may be necessary to remove the tumor or your adrenal glands.

Seek Help Today!

Call Urology of Greater Atlanta or request an appointment online if you have any concerns about adrenal cancer. You can book a visit with any of our board-certified urologists at one of our multiple locations throughout Georgia. We look forward to helping you get the peace of mind you deserve!