Unilateral adrenalectomy can be an alternative therapy for infantile onset Cushing' s syndrome caused by ACTHindependent macronodular adrenal hyperplasia with McCune-Albright syndrome

Hamajima, Takashi; Maruwaka, Kaori; Homma, Keiko; Matsuo, Kumihiro; Fukui, Kenji; Hasegawa, Tomonobu

doi:10.1507/endocrj.k10e-003

Cited by 19 publications

(13 citation statements)

References 14 publications

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“…The cases of 7 patients were previously reported. 1,5,6,14,17,36 Additional clinical information, pathologic material, and follow-up in 6 of these cases were generously supplied by reporting investigators, the patients’ physicians, or the patients’ families. The tenth patient, 23 a male infant with Cushing syndrome only, was included in the study because he had the same adrenal pathology as patients with McCune-Albright syndrome.…”

Section: Methodsmentioning

confidence: 99%

“…21,29 In rare cases, the cause of hypercortisolism was apparently in 1 adrenal gland because Cushing syndrome resolved after unilateral adrenalectomy. 14,32 Exceptionally, Cushing syndrome resolved spontaneously. 13 …”

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confidence: 98%

“…The anatomic basis of the Cushing syndrome has been attributed variously to bilateral nodular hyperplasia, 18 bilateral “atypical” adenomas, 5 bilateral macronodular hyperplasia, 14 and bilateral hyperplasia. 21,29 In rare cases, the cause of hypercortisolism was apparently in 1 adrenal gland because Cushing syndrome resolved after unilateral adrenalectomy.…”

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Primary Bimorphic Adrenocortical Disease

Carney¹,

Young

Stratakis

2011

American Journal of Surgical Pathology

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McCune-Albright syndrome (polyostotic fibrous dysplasia, café-au-lait skin spots, and precocious puberty) is a genetically mosaic disorder with populations of mutant and normal cells in affected organs. Cushing syndrome, a rare feature of the condition, usually affects infants and is the result of corticotropin-independent primary bilateral adrenal disease, usually interpreted as nodular adrenocortical hyperplasia. In this study of 9 patients with Cushing syndrome and McCune-Albright syndrome, light microscopy revealed a characteristic bimorphic pattern of diffuse and nodular hyperplasia and a distinctive form of cortical atrophy with apparent zona glomerulosa hyperplasia in 8 patients, all very young. The pattern could be explained by the presence of a mosaic distribution of mutant and normal cells in the adrenal glands. The findings are different from those in inherited or other forms of genetically caused Cushing syndrome. The ninth patient, aged 17 years, had an adrenal adenoma and diffuse cortical hyperplasia in each adrenal gland.

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Primary Bimorphic Adrenocortical Disease

Carney¹,

Young

Stratakis

2011

American Journal of Surgical Pathology

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“…However, several cases of MAS with a mild form of Cushing syndrome have spontaneously resolved [5, 6]. Recently, unilateral adrenalectomy has been reported to be successful in adult cases; however, the recurrence risk seemed considerably high [7-10], and only one pediatric case has been reported with a favorable prognosis [11]. …”

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confidence: 99%

Three-Quarters Adrenalectomy for Infantile-Onset Cushing Syndrome due to Bilateral Adrenal Hyperplasia in McCune-Albright Syndrome

et al. 2017

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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.

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“…This treatment has the great advantage of not requiring hormone replacement therapy (3). It was further reported that in cases in which the left and right adrenal glands differed in size, unilateral adrenalectomy of the larger adrenal gland was more effective (12).…”

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Usefulness and Limitations of Unilateral Adrenalectomy for ACTH-independent Macronodular Adrenal Hyperplasia in a Patient with Poor Glycemic Control

et al. 2012

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Adrenocorticotropic hormone (ACTH)-independent macronodular adrenal hyperplasia (AIMAH) is a rare disease which causes Cushing's syndrome. Bilateral adrenalectomy has been recommended as the treatment of choice for AIMAH. However, bilaterally adrenalectomized patients require lifelong steroid replacement therapy. Therefore, an increasing number of patients have undergone unilateral adrenalectomy for AIMAH. We report a case of AIMAH due to refractory diabetes in whom unilateral adrenalectomy initially yielded good diabetes control, but in whom poor glycemic control developed after 5 years, requiring eventual additional contralateral adrenalectomy. In elderly patients with AIMAH, one-stage bilateral adrenalectomy may be the treatment of choice.

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Unilateral adrenalectomy can be an alternative therapy for infantile onset Cushing' s syndrome caused by ACTHindependent macronodular adrenal hyperplasia with McCune-Albright syndrome

Cited by 19 publications

References 14 publications

Primary Bimorphic Adrenocortical Disease

Primary Bimorphic Adrenocortical Disease

Three-Quarters Adrenalectomy for Infantile-Onset Cushing Syndrome due to Bilateral Adrenal Hyperplasia in McCune-Albright Syndrome

Usefulness and Limitations of Unilateral Adrenalectomy for ACTH-independent Macronodular Adrenal Hyperplasia in a Patient with Poor Glycemic Control

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