Table 2.

Treatment, Adrenal Pathological Diagnosis, Duration of Follow-up, and Status of 17 Patients with Cushing Syndrome and Carney Complex

Patient	Treatment	Pathological Diagnosis		Duration of Follow-up, y	Status
		Initial (Year)	Study
1	None	Cortical hyperplasia (1937)	PPNAD with cortical atrophy (autopsy)	60	Dead at 77 y No CS Adrenal sufficient Autopsy: metastatic pancreatic carcinoma, bilateral PPNAD, pituitary normal
2	None	No adrenal tissue obtained at presentation (1937)	PPNAD with cortical atrophy (autopsy)	27	Dead at 44 y (sudden) Chronic Cushing syndrome Autopsy: severe coronary atherosclerosis, bilateral PPNAD, pituitary normal, testes grossly normal
3	Bilateral adrenalectomy	PPNAD	PPNAD with cortical atrophy	27	Alive at 67 y Well Adrenal insufficient
4	Subtotal (<90%) left adrenalectomy Biopsy right adrenal	Left adrenal: diffuse cortical hyperplasia Right adrenal: normal cortex	PPNAD variant	66	Alive at 68 y Depression Adrenal sufficient Osteopenia Type 2 diabetes mellitus 4×4×7-mm thyroid nodule
5	Bilateral adrenalectomy	Right adrenal: single cortical nodule Left adrenal: nodular hyperplasia	PPNAD variant	4	Well at 11 y Adrenal insufficient
6	Bilateral adrenalectomy, asynchronous (ages 4 and 13 y)	Consistent with PPNAD	PPNAD variant	6	Well at 19 y Adrenal insufficient
7	Bilateral adrenalectomy	Consistent with PPNAD	PPNAD variant PPNAD in adrenal ectopia	5	Well at 21 y Adrenal insufficient
8	Bilateral adrenalectomy	Micronodular hyperplasia	PPNAD variant	5	Well at 17 y Adrenal insufficient
9	Bilateral adrenalectomy	PPNAD	PPNAD	23	Alive at 65 y Adrenal insufficient Severe tricuspid valve regurgitation due to Ebstein anomaly
10	Bilateral adrenalectomy	PPNAD	PPNAD with 1.8-cm epicapsular myelolipoma	29	Well at 63 y Adrenal insufficient
11	Left: total adrenalectomy Right: subtotal (67%–75%) adrenalectomy	Cortical hyperplasia	Suggestive of PPNAD	54	Alive at 61 y Adrenal sufficient Cervical nodal and pulmonary metastatic follicular thyroid carcinoma
12	Bilateral adrenalectomy	PPNAD	PPNAD	14	Well at 33 y Adrenal insufficient
13	Bilateral adrenalectomy, asynchronous (ages 9 and 18 y)	Left adrenal (1969): pigmented carcinoma Right adrenal (1973): adenomatous cortical hyperplasia	PPNAD with vacuolated cell hyperplasia and massive intracapsular and epicapsular cortical hyperplasia	42	Alive at 59 y Adrenal insufficient Mitral and tricuspid regurgitation after 5 cardiac operations for cardiac myxoma
14	Left total and right subtotal (67%) adrenalectomy (age 10 y) Completion of right adrenalectomy (age 18 y)	Cortical hyperplasia	PPNAD with vacuolated cell hyperplasia and massive epicapsular cortical hyperplasia	44	Alive at 63 y Adrenal insufficient Hemiplegia due to cardiac myxoma embolus
15	Bilateral adrenalectomy (age, 46 y)	Consistent with PPNAD	PPNAD	2	Well at 48 y Adrenal insufficient
16	Bilateral adrenalectomy (age, 12 y)	PPNAD	PPNAD with 1.5-cm macronodule	1	Well at 17 y Adrenal insufficient
17	Bilateral adrenalectomy, asynchronous (right at age 2 y and left at 29 y)	Right adrenal (1985): cortical adenoma Left adrenal (2012): consistent with PPNAD	Right: cortical adenoma (2 cm); rare PPNAD-type micronodules; outer cortex synaptophysin positive Left: PPNAD	27 (after right adrenalectomy) 3 (after left adrenalectomy)	Dead at 33 y (sudden) Adrenal insufficient Autopsy: no anatomical cause of death found; brain, mesial temporal sclerosis; pituitary, normal; vitreous body electrolytes, normal; kidneys, nephrosclerosis; vertebral bone, nonspecific osteopenia

Abbreviation: PPNAD, primary pigmented nodular adrenocortical disease.