Case No. Sex Age (yr) at first surgery Manifestations of Cushing’s syndrome (CS) Computed tomography findings Adrenal scintigraphy* Adrenalectomy Pathological findings of adrenal glands Features of Carney complex Gene mutation II-2 Male   42 Overt CS No remission of CS after right total adrenalectomy Right tiny adrenal nodule, left adrenal nodule (2 cm) Bilateral adrenal uptake Bilateral total adrenalectomy Bilateral diffuse PPNAD PPNAD, bilateral papillary thyroid  carcinoma PRKAR1A?s (c.46 C>T) III-2 Female   24 Three stages of CS‡ on different dates Remission of CS for >11 years after left total adrenalectomy Left adrenal nodule (3 x 2 cm), normal-sized right adrenal gland Left adrenal uptake Left total adrenalectomy, right subtotal adrenalectomy (80%) Left focal PPNAD, right diffuse PPNAD PPNAD, bilateral thyroid cysts PRKAR1A? (c.46 C>T) III-4 Male   32§ Latent CS Not done Not done No Unknown No screened PRKAR1A? (c.46 C>T) II-4 Male   45 Overt CS Persistent remission of CS for >12 years after left total adrenalectomy Left adrenal nodule (2 cm), normal-sized right adrenal gland Earlier uptake of left adrenal than right Left total adrenalectomy Left diffuse PPNAD PPNAD, bilateral thyroid cysts PRKAR1A? (c.46 C>T) III-7 Male   29 Overt CS No remission of CS after left total adrenalectomy Normal-sized bilateral adrenal glands Bilateral adrenal uptake Left total adrenalectomy Left diffuse PPNAD PPNAD PRKAR1A ? (c.46 C>T)

Table 2: Clinical characteristics in a family with primary pigmented nodular adrenocortical disease (PPNAD). *Adrenal scintigraphy: 131I-6- iodomethyl-19-norcholesterol adrenal scintigraphy. ?PRKAR1A: regulatory subunit R1A of protein kinase A. ‡Three stages of CS: overt, subclinical, and latent Cushing’s syndrome. 32: The patient was 32 years old when the gene study was performed in October 2011.