Carney Complex: A Rare Case of Multicentric Cardiac Myxoma Associated with Endocrinopathy

Table 1

Diagnostic criteria for carney complex.

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(1) Spotty skin pigmentation with a typical distribution (lips, conjunctiva and inner or outer canthi, vaginal and penile mucosa)

(2) Myxoma (cutaneous and mucosal)^a

(3) Cardiac myxoma^a

(4) Breast myxomatosis^a or fat-suppressed magnetic resonance imaging findings suggestive of this diagnosis

(5) PPNAD^a or paradoxical positive response of urinary glucocorticosteroids to dexamethasone administration during Liddle’s test

(6) Acromegaly due to GH-producing adenoma^a

(7) LCCSCT^a or characteristic calcification on testicular ultrasonography

(8) Thyroid carcinoma^a or multiple, hypoechoic nodules on thyroid ultrasonography, in a young patient

(9) Psammomatous melanotic schwannoma^a

(10) Blue nevus, epithelioid blue nevus (multiple)^a

(11) Breast ductal adenoma (multiple)^a

(12) Osteochondromyxoma^a

Supplemental criteria

(1) Affected first-degree relative

(2) Inactivating mutation of the PRKARIA gene

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To make a diagnostic of CNC, a patient must either (1) exhibit two of the manifestations of the diseases listed or (2) exhibit one of these manifestations and meet one of the supplemental criteria (an affected first-degree relative or an inactivating mutation of the PRKARIA gene). ^aWith histologic information.