Carney Complex Detailed

Multiple endocrine neoplasia syndrome related to PRKAR1A.

Written by: Saygı Hospital Health Guide Editorial Board

Last updated: June 11, 2026

This content has been compiled by the Saygı Hospital Health Guide Editorial Board and is periodically reviewed by a specialist physician.

References (5)

This content is for informational purposes only and does not constitute medical advice. You can book an appointment at our Endokrinoloji department. Book Appointment →

What is Carney Complex Detailed?

Carney complex is an autosomal dominant multiple neoplasia syndrome usually caused by PRKAR1A (regulatory subunit of protein kinase A) gene mutations on chromosome 17q. It is characterised by primary pigmented nodular adrenocortical disease (PPNAD), cardiac myxoma and skin pigmentation.

Cardiac myxomas may be multifocal and recurrent (with high mortality risk). Other tumours include psammomatous melanotic schwannoma, large-cell calcifying Sertoli cell tumour, growth-hormone-producing pituitary adenoma and thyroid tumours.

Symptoms

Lentigines on the face, lips and conjunctiva (skin pigmentation)

Cushing syndrome (PPNAD)

Cardiac myxoma symptoms (embolism, syncope, sudden death)

Acromegaly findings (GH-secreting adenoma)

Testicular mass (LCCSCT)

Hearing loss/back pain (psammomatous melanotic schwannoma)

Risk Factors

PRKAR1A mutation (autosomal dominant)

Family history of Carney complex

Spotty pigmentation observed in childhood

Cardiac myxoma at young age

First-degree relative diagnosis

When to See a Doctor?

If you experience any of the following symptoms, seek medical attention promptly:

Spotty skin pigmentation + endocrine findings
Family history of cardiac myxoma at young age
Cushing syndrome unexplained by adrenal mass
Multiple endocrine tumour combination
First-degree relative with PRKAR1A mutation

Treatment Methods

PRKAR1A genetic testing

Annual cardiac echocardiography (myxoma screening)

Annual cortisol/UFC, dexamethasone test (PPNAD)

Pituitary MR + IGF-1 (GH adenoma)

Testicular ultrasound (LCCSCT)

Thyroid ultrasound (annual)

Surgical resection (myxoma excision, bilateral adrenalectomy)

Family screening (first-degree relatives)

Lifelong multidisciplinary follow-up

Which Department to Visit?

You can visit our Endokrinoloji department for these complaints. Our specialist physicians will create the most suitable treatment plan for you.

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Let us help you

You can make an appointment with our specialists or contact us for your concerns.

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Health Disclaimer: The information on this page is prepared for general informational purposes only. It does not replace medical diagnosis and treatment. Please consult your physician for your complaints. Saygı Hospital does not accept responsibility for actions taken based on the information on this page.