Cushing’s syndrome due to autonomous macronodular adrenal hyperplasia: long-term follow-up after unilateral adrenalectomy

Boronat, Mauro; Lucas, T.; Barceló, B; Alameda, C.; Hotait, Hassan; Estrada, Javier

doi:10.1136/pgmj.72.852.614

Cited by 18 publications

(7 citation statements)

References 10 publications

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“…Bilateral adrenalectomy via an overt or laparoscopic approach is the most useful treatment for patients with AIMAH and hormonal hypersecretion ( 21 , 22 ). However, in patients exhibiting moderately increased hormonal production, unilateral adrenalectomy is proposed as a safe and effective alternative; it is expected that as the cell mass increases in the contralateral adrenal gland, a second adrenalectomy may be required ( 23 , 24 ).…”

Section: Discussionmentioning

confidence: 99%

Diagnosis and treatment of adrenocorticotrophic hormone-independent macronodular adrenocortical hyperplasia: A report of 23 cases in a single center

Yang

2014

Experimental and Therapeutic Medicine

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The aim of the present study was to present the treatment observations of patients with adrenocorticotrophic hormone (ACTH)-independent macronodular adrenal hyperplasia (AIMAH) from a single medical center. A total of 23 patients with AIMAH were retrospectively reviewed according to the clinical features of the patient. Physical symptoms and biochemical evidence of Cushing’s syndrome (CS) were present in all the cases. High and low dose dexamethasone failed to suppress cortisone secretion on the suppression tests and ACTH levels were low in all the cases. Bilateral enlarged adrenal glands were observed with computed tomography scans in the 23 cases, which were determined to be bilateral adrenal macronodular hyperplasia by pathological analysis. Normal blood pressure was observed in eight patients in the 2–8 years following the bilateral adrenalectomy. A total of 15 cases underwent single side adrenalectomy and their blood pressures markedly decreased three years following surgery; medical treatment was administered to 12 of the 15 patients, while three patients underwent a contralateral adrenalectomy. Nelson’s syndrome was not diagnosed for any of the cases. AIMAH has unique endocrinological, radiological and pathological features, presenting as an independent etiological factor of CS. Diagnosis of AIMAH is usually derived from pathological examination and long term remission may be achieved by unilateral adrenalectomy. Contralateral adrenalectomy should be performed in cases of recurrence, when followed with periodical examination of the symptoms and the serum concentration of cortisol.

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Section: Discussionmentioning

confidence: 99%

Diagnosis and treatment of adrenocorticotrophic hormone-independent macronodular adrenocortical hyperplasia: A report of 23 cases in a single center

Yang

2014

Experimental and Therapeutic Medicine

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“…All of them presented with Table 1. A detailed case report of patient 1 has been published previously (9). Unilateral adrenalectomy of the largest gland was performed in all four patients.…”

Section: Subjectsmentioning

confidence: 99%

Is unilateral adrenalectomy an alternative treatment for ACTH-independent macronodular adrenal hyperplasia?: Long-term follow-up of four cases

Lamas

Alfaro

Lucas

et al. 2002

European Journal of Endocrinology

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Objective: ACTH-independent macronodular adrenal hyperplasia is a rare cause of Cushing's syndrome. Bilateral adrenalectomy is considered the treatment of choice, but the patient is obliged to receive lifetime steroid replacement therapy and is susceptible to adrenal insufficiency crisis. New therapeutic alternatives are being proposed as new etiopathological features of the disease are known. Unilateral adrenalectomy of the largest gland can be a safe and effective alternative, but only short-term follow-up is reported in the literature. We present four consecutive patients with ACTH-independent macronodular hyperplasia and long-term remission of Cushing's syndrome after unilateral adrenalectomy. Subjects: Four consecutive patients (two males and two females, mean age 50.3 years) with Cushing's syndrome due to ACTH-independent macronodular adrenal hyperplasia underwent unilateral adrenalectomy of the largest gland. Results: The weight of the resected glands ranged from 26.8 to 210 g. Two patients suffered transient post-surgical adrenal insufficiency and had steroid replacement therapy for 60 and 14 months respectively. After a mean follow-up of 78.8 months (range 30-137 months) all the patients persist without any evidence of Cushing's syndrome. Urinary free cortisol and serum cortisol, after the adrenal insufficiency stage, have always stayed within their normal ranges, but cortisol circadian rhythm and suppressibility after dexamethasone have never normalized. No further enlargement of the contralateral gland has been documented 62 to 126 months after surgery in three of the four patients. Conclusions: Unilateral adrenalectomy can be an effective and safe alternative treatment for ACTH-independent macronodular adrenal hyperplasia, and can achieve long-term remission of Cushing's syndrome.

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“…Bilateral adrenalectomy has been the most utilized treatment in patients with AIMAH and hormonal hypersecretion including overt CS [2,4,6]. However, in patients with moderately increased hormonal production, unilateral adrenalectomy has been proposed as a safe and effective alternative [75,[89][90][91]; it is expected that, as the cell mass increases in the contralateral adrenal, a second adrenalectomy may become necessary later. AIMAH is a benign process that has never been shown to become malignant.…”

Section: Treatment Of Patients With Aimahmentioning

confidence: 99%

ACTH-Independent Macronodular Adrenal Hyperplasia

et al. 2011

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ACTH-independent macronodular adrenal hyperplasia (AIMAH) is an uncommon cause of Cushing's syndrome (CS). The pathophysiology of this disorder is heterogeneous in its molecular origin and also in its clinical presentation. AIMAH can present mainly as an incidental radiological finding with sub-clinical CS or rarely with overt CS. In a few familial cases reported with AIMAH, specific aberrant G-protein coupled receptors were expressed in the adrenals of all affected members, but sporadic cases are more common. The aberrant adrenal function of G-protein coupled receptors can lead to cell proliferation and abnormal regulation of steroidogenesis. Unilateral or bilateral adrenalectomy has been the most frequently used treatment for this adrenal disorder; alternatively, the identification of aberrant receptors using in vivo protocol of investigation can offer specific pharmacological approach to control abnormal steroidogenesis and possibly prevent AIMAH progression. (Endocrinol Metab 26:1-11, 2011)

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Cushing’s syndrome due to autonomous macronodular adrenal hyperplasia: long-term follow-up after unilateral adrenalectomy

Abstract: Summary This report describes a case of Cushing’s syndrome due to autonomous macronodular adrenocortical hyperplasia in which unilateral resection of the right adrenal resolved the Cushing’s syndrome.

Cited by 18 publications

References 10 publications

Diagnosis and treatment of adrenocorticotrophic hormone-independent macronodular adrenocortical hyperplasia: A report of 23 cases in a single center

Diagnosis and treatment of adrenocorticotrophic hormone-independent macronodular adrenocortical hyperplasia: A report of 23 cases in a single center

Is unilateral adrenalectomy an alternative treatment for ACTH-independent macronodular adrenal hyperplasia?: Long-term follow-up of four cases

ACTH-Independent Macronodular Adrenal Hyperplasia

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