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Table 1 Main features and diagnostic criteria of Carney complex. This table lists the most frequent features of CNC and their estimated frequency [according to references 1, 3–5, 8 and our personal observations]. The incidence of each manifestation depends on its presentation and might not reflect true prevalence. For instance, according to autopsy studies PPNAD is a constant feature in CNC patients [8], however, reports of Cushing syndrome in the literature indicate that only 25 to 45 % of CNC patients have PPNAD and our personnal experience suggests that about 60 % of CNC patients systematically investigated have PPNAD. It has been established that at least two of these manifestations (except ovrian cyst) need to be present to confirm the diagnosis of CNC. If the patient has a germline PRKAR1A mutation and/or a first-degree relative affected by CNC, a single manifestation is sufficient for the diagnosis.

From: Carney complex (CNC)

Main features of Carney complex (%)
Primary Pigmented Nodular Adrenocortical Disease (PPNAD) 25–60
Cardiac myxoma 30–60
Skin myxoma 20–63
Lentiginosis 60–70
Multiple blue nevus  
Breast ductal adenoma 25
Testicular tumors (LCCSCT: Large-Cell Calcifying Sertoli Cell Tumor) (in male) 33–56
Ovarian cyst (in female) 20–67
Acromegaly 10
Thyroid tumor 10–25
Melanotic schwannoma 8–18
Osteochondromyxoma <10