Background: Bilateral adrenalectomy is performed in cases with infantile-onset Cushing syndrome due to bilateral adrenal hyperplasia in McCune-Albright syndrome (MAS) because severe Cushing syndrome with heart failure and liver dysfunction can have a lethal outcome. This procedure can completely ameliorate hypercortisolism, although lifetime steroid replacement therapy and steps to prevent adrenal crisis are necessary. Recently, the efficacy of unilateral adrenalectomy has been reported in adult cases of bilateral macronodular adrenal hyperplasia, but there is no consensus regarding the appropriate surgical treatment for bilateral adrenal hyperplasia in MAS. Objective: A 6-month-old girl presented with café-au-lait spots, short stature, central obesity, a moon face, and hypertension. Endocrinological tests and imaging studies led to the diagnosis of ACTH-independent Cushing syndrome due to bilateral adrenal hyperplasia induced by MAS. “Three-quarters adrenalectomy”, namely right-sided total adrenalectomy and left-sided half adrenalectomy, was carried out. An activating mutation of the GNAS1 gene (p.Arg201Cys) was identified in the adrenal tissues. Since the operation, our patient has been in a state of clinical remission for more than 2 years. Conclusion: Our original surgical intervention, three-quarters adrenalectomy, may be a new treatment option for Cushing syndrome associated with MAS.

1.
McCune D, Bruch H: Osteodystrophia fibrosa: report of a case in which the condition was combined with precocious puberty, pathologic pigmentation of the skin and hyperthyroidism, with a review of the literature. Am J Dis Child 1937; 54: 806–848.
2.
Albright F, Butler AM, Hampton AO, Smith P: Syndrome characterized by osteitis fibrosa disseminata, areas of pigmentation and endocrine dysfunction, with precocious puberty in females. N Engl J Med 1937; 216: 727–746.
3.
Volkl TM, Dorr HG: McCune-Albright syndrome: clinical picture and natural history in children and adolescents. J Pediatr EndocrinolMetab 2006; 19(suppl 2):551–559.
4.
Weinstein LS, Shenker A, Gejman PV, Merino MJ, Friedman E, Spiegel AM: Activating mutations of the stimulatory G protein in the McCune-Albright syndrome. N Engl J Med 1991; 325: 1688–1695.
5.
Brown RJ, Kelly MH, Collins MT: Cushing syndrome in the McCune-Albright syndrome. J Clin Endocrinol Metab 2010; 95: 1508–1515.
6.
Collins MT, Singer FR, Eugster E: McCune-Albright syndrome and the extraskeletal manifestations of fibrous dysplasia. Orphanet J Rare Dis 2012; 7(suppl 1):S4.
7.
Li J, Yang CH: Diagnosis and treatment of adrenocorticotrophic hormone-independent macronodular adrenocortical hyperplasia: a report of 23 cases in a single center. Exp Ther Med 2015; 9: 507–512.
8.
Debillon E, Velayoudom-Cephise FL, Salenave S, Caron P, Chaffanjon P, Wagner T, Massoutier M, Lambert B, Benoit M, Young J, Tabarin A, Chabre O: Unilateral adrenalectomy as a first-line treatment of Cushing’s syndrome in patients with primary bilateral macronodular adrenal hyperplasia. J Clin Endocrinol Metab 2015; 100: 4417–4424.
9.
Xu Y, Rui W, Qi Y, Zhang C, Zhao J, Wang X, Wu Y, Zhu Q, Shen Z, Ning G, Zhu Y: The role of unilateral adrenalectomy in corticotropin-independent bilateral adrenocortical hyperplasias. World J Surg 2013; 37: 1626–1632.
10.
Albiger NM, Ceccato F, Zilio M, Barbot M, Occhi G, Rizzati S, Fassina A, Mantero F, Boscaro M, Iacobone M, Scaroni C: An analysis of different therapeutic options in patients with Cushing’s syndrome due to bilateral macronodular adrenal hyperplasia: a single-centre experience. Clin Endocrinol (Oxf) 2015; 82: 808–815.
11.
Hamajima T, Maruwaka K, Homma K, Matsuo K, Fujieda K, Hasegawa T: Unilateral adrenalectomy can be an alternative therapy for infantile onset Cushing’ s syndrome caused by ACTH-independent macronodular adrenal hyperplasia with McCune-Albright syndrome. Endocr J 2010; 57: 819–824.
12.
Nieman LK, Biller BM, Findling JW, Murad MH, Newell-Price J, Savage MO, Tabarin A, Endocrine S: Treatment of Cushing’s syndrome: an Endocrine Society Clinical Practice guideline. J Clin Endocrinol Metab 2015; 100: 2807–2831.
13.
Iacobone M, Albiger N, Scaroni C, Mantero F, Fassina A, Viel G, Frego M, Favia G: The role of unilateral adrenalectomy in ACTH-independent macronodular adrenal hyperplasia (AIMAH). World J Surg 2008; 32: 882–889.
14.
Breault L, Chamoux E, Lehoux JG, Gallo-Payet N: Localization of G protein alpha-subunits in the human fetal adrenal gland. Endocrinology 2000; 141: 4334–4341.
15.
Insel PA, Zhang L, Murray F, Yokouchi H, Zambon AC: Cyclic AMP is both a pro-apoptotic and anti-apoptotic second messenger. Acta Physiol (Oxf) 2012; 204: 277–287.
16.
Gillis D, Rosler A, Hannon TS, Koplewitz BZ, Hirsch HJ: Prolonged remission of severe Cushing syndrome without adrenalectomy in an infant with McCune-Albright syndrome. J Pediatr 2008; 152: 882–884, 884.e1–e4.
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