What is Carney Complex?

Carney Complex is a rare genetic syndrome characterized by multiple tumors (cardiac myxomas, skin pigmentation, and various endocrine tumors). "LAMB" or "NAME" syndrome are older names for this condition.

Major Features:

• Cardiac Myxomas (30-60%): Benign heart tumors; can cause embolism, heart failure, sudden death
• Skin Pigmentation (70-90%): Lentigines (brown spots) on face, lips, conjunctiva, genitals
• Primary Pigmented Nodular Adrenocortical Disease (PPNAD) (25-60%): Cushing syndrome from bilateral adrenal nodules
• Pituitary Adenomas (10-20%): GH-secreting → acromegaly/gigantism
• Thyroid Nodules/Cancer
• Testicular Tumors (Sertoli Cell): In males
• Ovarian Cysts
• Schwannomas (Psammomatous Melanotic): Nerve tumors

Endocrine Manifestations:

• Cushing Syndrome (25-60%): From PPNAD; often cyclical, ACTH-independent
• Acromegaly/Gigantism (10-20%): Pituitary GH excess
• Thyroid Disease

Genetics & Diagnosis:

• PRKAR1A Gene Mutation: Autosomal dominant
• Diagnosis: Clinical criteria (≥2 major features) or family history + 1 feature + genetic testing
• Screening: Echocardiogram (cardiac myxomas), hormone testing, imaging

Treatment:

• Cardiac Myxomas: Surgical removal (can recur)
• PPNAD/Cushing's: Bilateral adrenalectomy
• Pituitary Adenomas: Surgery, somatostatin analogs
• Lifelong Surveillance: Annual echocardiograms, hormone screening