Although there is currently no way to prevent tumors from growing in Carney complex, regular monitoring with imaging and other tests can help identify problems early so that treatment can begin.
Treatments for symptoms of Carney complex include medication and surgery.1,2
If a tumor is not causing any symptoms, as is usually the case with thyroid adenomas, treatment may not be needed.
Tumors that cause only mild symptoms or that cause hormone imbalances, such as pituitary tumors, may be treatable with medication. However, for cardiac tumors and tumors on endocrine glands, surgery to remove the tumors may be the best option. A combination of treatments may also be effective.
In the rare instances that a tumor develops into cancer, also called a carcinoma, surgery is usually required. Radiation and medication are also used to treat carcinomas.
The Importance of Ongoing Screening in Carney Complex
In Carney complex, tumors can grow at any time. Therefore, ongoing screening for new tumors is an important part of standard care for someone diagnosed with Carney complex. The type of screening test and the frequency differ slightly depending on the age of the person when they are diagnosed.
Healthcare providers suggest the following screening tests and frequency. Please note that these suggestions are general and should not be interpreted as care recommendations. If you have specific questions about Carney complex in yourself or your child, please consult a healthcare provider.
Before Puberty
| Test | Frequency | To Detect |
|---|---|---|
| Growth rate monitoring | Yearly |
|
| Pubertal staging; blood tests to measure cortisol, growth hormone, and estrogen/testosterone | Yearly |
|
| Echocardiogram (echo) | Every 6 months; more often if surgery to remove a cardiac myxoma has already occurred | Cardiac myxoma |
| Testicular ultrasound (males only) | Yearly | Large-cell calcifying Sertoli cell tumors |
| Ovarian ultrasound (females only) | Yearly | Ovarian cysts or adenomas |
After Puberty
| Test | Frequency | To Detect |
|---|---|---|
| Echocardiogram (ECHO) | Yearly; more often if surgery to remove a cardiac myxoma has already occurred | Cardiac myxoma |
| Testicular ultrasound (males only) | Yearly | Large-cell calcifying Sertoli cell tumors |
| Ovarian ultrasound (females only) | Baseline; then as needed | Ovarian cysts or adenomas |
| Thyroid ultrasound | Baseline; then as needed | Thyroid nodules and adenomas |
| Urinary free cortisol level test | Yearly | |
| Serum IGF-1 test | Yearly |
|
| Diurnal cortisol level test | Baseline; then as needed |
|
| Dexamethasone stimulation test (sometimes called Liddle test) | Baseline; then as needed |
|
| Adrenal CT scan | Baseline; then as needed | Adrenal tumors |
| Pituitary MRI scan | As needed | Pituitary tumors |
| Oral glucose tolerance test | As needed | Pituitary tumors |
| Serum growth hormone and prolactin test | Baseline; then as needed | Pituitary tumors |
| MRI scan of brain, spine, chest abdomen, retroperitoneum, or pelvis | Baseline; repeat if clinical neurological signs suggest possibility | Schwannomas (tumors on the Schwann cells) |
Citations
- National Organization for Rare Disorders. (2018). Carney Complex. Retrieved November 11, 2021, from https://rarediseases.org/rare-diseases/carney-complex/
- Stratakis, C. A., & Raygada, M. (2003 Feb 5 [Updated 2018 Aug 16]). Carney Complex. In: Adam, M. P., Ardinger, H. H., Pagon, R. A., et al., eds. GeneReviews® [Internet]. Seattle (WA): University of Washington. Retrieved June 15, 2022, from https://www.ncbi.nlm.nih.gov/books/NBK1286/
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