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Carney complex
Source: Childhood Soft Tissue Sarcoma Treatment (PDQ®)–Health Professional Version, National Cancer Institute.
Source updated: April 21, 2025 · Captured 2026-09-09.
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Context: Treatment of Tumors of Uncertain Differentiation / Myxoma NOS
Carney complex is an autosomal dominant syndrome caused by variants in the PRKAR1A gene, located on chromosome 17.[1] The syndrome is characterized by cardiac and cutaneous myxomas, pale brown to brown lentigines, blue nevi, primary pigmented nodular adrenocortical disease causing Cushing syndrome, and a variety of endocrine and nonendocrine tumors, including pituitary adenomas, thyroid tumors, and large cell calcifying Sertoli cell tumor of the testis.[1-3] There are published surveillance guidelines for patients with Carney complex that include cardiac, testicular, and thyroid ultrasonography.
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For patients with the Carney complex, prognosis depends on the frequency of recurrences of cardiac and skin myxomas and other tumors.
For more information about the treatment of conditions related to Carney complex, see the following summaries:
Childhood Cardiac Tumors Treatment.
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Childhood Testicular Cancer Treatment.
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Childhood Thyroid Cancer Treatment.
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Publication references
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Preserved source evidence · Not medical advice