Carney Complex

A rare autosomal dominant disease characterized by skin pigmentation, myxomas and endocrine tumors.

Written by: Saygı Hospital Health Guide Editorial Board

Last updated: June 10, 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?

Carney complex is a rare autosomal dominant multiple endocrine neoplasia syndrome caused mostly by mutations in the PRKAR1A gene. It produces a characteristic triad of skin pigmentation (freckling), myxomas, and endocrine overactivity.

Cardiac myxomas can occur in any chamber and at multiple sites; they may cause embolism, valve dysfunction or sudden death. Cushing syndrome develops as primary pigmented nodular adrenocortical disease (PPNAD).

Other features include large-cell calcifying Sertoli cell tumors of the testis, growth hormone-secreting pituitary adenoma, thyroid nodules, and schwannomas. Genetic counseling and lifelong cardiac and endocrine surveillance are required.

Symptoms

Skin freckling (lentigines) and blue nevi

Cardiac myxoma symptoms (dyspnea, syncope)

Cushingoid appearance

Acromegalic features

Testicular masses

Cutaneous myxomas

Thyroid nodules

Risk Factors

PRKAR1A gene mutation

Family history

Autosomal dominant inheritance

PRKACA/PRKACB mutations (rare)

First-degree relatives

Carney complex history

When to See a Doctor?

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

Newly noticed widespread freckling
Cardiac symptoms (palpitation, syncope)
Cushing syndrome findings
Acromegalic features
Testicular mass
Family history with Carney complex

Treatment Methods

Cardiac myxoma resection

Adrenalectomy (in PPNAD-related Cushing)

Pituitary adenoma surgery

Testicular tumor follow-up/surgery

Annual cardiac MRI/echocardiography

Genetic counseling and family screening

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.