Challenges in the Diagnostic Work-Up and Management of Patients with Subclinical Cushing's Syndrome and Bilateral Adrenal Masses

Maghrabi, Adam H.; Yaqub, Abid; Denning, Krista L.; BenHamed, Nesreen; Faiz, Saba; Saleem, Tipu Faiz

doi:10.4158/ep12277.ra

Cited by 22 publications

(19 citation statements)

References 22 publications

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“…ACTH-independent CS is occasionally caused by bilateral adrenocortical lesions, including unilateral functional adenoma with a contralateral non-functional mass, bilateral ACTH-independent macronodular adrenal hyperplasia (AIMAH), bilateral primary pigmented nodular adrenocortical disease (PPNAD), and an extremely rare entity, bilateral adrenocortical tumors [ 3 ]. Determining the nature and function of bilateral adrenal masses is always a challenge in clinical practice [ 4 , 5 ]. We herein report a new case of a Chinese female patient with ACTH-independent Cushing’s syndrome due to bilateral cortisol-secreting adenomas, which was diagnosed through adrenal venous sampling (AVS) adjusted by plasma aldosterone and subsequently confirmed by postoperative pathology.…”

Section: Introductionmentioning

confidence: 99%

ACTH-independent Cushing’s syndrome with bilateral cortisol-secreting adrenal adenomas: a case report and review of literatures

Wang

Liu

et al. 2018

BMC Endocr Disord

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BackgroundAdrenocorticotropic hormone (ACTH)-independent Cushing’s syndrome (CS) with bilateral cortisol-secreting adenomas has been rarely reported in the literatures. Precise recognition and management of this disorder constitute a challenge to clinicians due to the difficulty of exact location of the functional lesions.Case presentationWe herein report a new case of a Chinese female patient with a complaint of exertional dyspnea for over 10 years. ACTH-independent CS was diagnosed based on undetectable ACTH and unsuppressed cortisol levels by dexamethasone. Computed tomography (CT) scan indicated bilateral adrenal masses, and adrenal venous sampling (AVS) adjusted by plasma aldosterone revealed hypersecretion of cortisol from both adrenal glands. Bilateral cortisol-secreting adrenal adenomas were suspected and confirmed by the postoperative pathology in subsequent two-step bilateral laparoscopic adrenalectomy. The symptoms and signs of CS relieved after surgery with continuous glucocorticoid replacement.ConclusionsAVS adjusted by plasma aldosterone could be a useful technique in diagnosing ACTH-independent CS with bilateral adrenal adenomas prior to surgery. And the aldosterone ratio could be used to confirm the success of adrenal vein cannulation in this situation.

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Section: Introductionmentioning

confidence: 99%

ACTH-independent Cushing’s syndrome with bilateral cortisol-secreting adrenal adenomas: a case report and review of literatures

Wang

Liu

et al. 2018

BMC Endocr Disord

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“…Bilateral cortisol-secreting adenomas are a rare cause of CS, a condition that is predominantly seen in women of child-bearing age [2][3][4][5][6] as was the case in our patient. Making a differential diagnosis between cortisol-secreting adenomas and ACTH-independent macronodular adrenal hyperplasia (AIMAH) or unilateral cortisol-secreting adenoma with a contralateral nonfunctioning cortical adenoma is a challenge [12].…”

Section: Discussionmentioning

confidence: 99%

A case of Cushing’s syndrome due to bilateral cortisol-secreting adenomas with unilateral DHEAS oversecretion

et al. 2015

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Cortisol-seCreting adrenal adenomas account for 10-15% of endogenous Cushing's syndrome (CS) cases [1]. Bilateral cortisol-secreting adenomas, however, are extremely rare [2][3][4][5][6]. In contrast to ACTHdependent CS and adrenocortical-carcinomas, adrenocortical adenomas generally (with rare exception [7]) only secrete glucocorticoids without co-secretion of adrenal androgens [8]. We herein report a rare case of CS due to bilateral cortisol-secreting adrenal adenomas (one on the left and two on the right) with unilateral (left-sided) dehydroepiandrosterone sulphate (DHEAS) oversecretion. This is a unique hormonal Abstract. A 39-year-old Japanese woman presented with typical clinical symptoms of Cushing's syndrome, including amenorrhea and hirsutism, for 2 years. The results of her initial endocrine evaluation were consistent with ACTHindependent Cushing's syndrome due to bilateral adrenal masses (diameter of 3.1 cm and 2.4 cm on the left and right, respectively). Serum dehydroepiandrosterone levels were 6,901 ng/mL (normal range 230-2,660 ng/mL). Bilateral laparoscopic adrenalectomies were performed (left adrenalectomy first and right adrenalectomy 3 months later). Following the left adrenalectomy, the results of the endocrine evaluations were still consistent with a diagnosis of ACTH-independent Cushing's syndrome. Serum dehydroepiandrosterone sulphate levels, however, were below the normal range (143 ng/mL). Unexpectedly, the patient's menstruation resumed 2.5 months after the left adrenalectomy. Pathological examination of the resected glands showed bilateral adrenocortical adenomas, one on the left with a diameter of 3 cm, and two on the right with diameters of 0.7 cm and 1.3 cm. Immunohistochemical analysis revealed side chain cleavage, 17α-hydroxylase, 3β-hydroxysteroid dehydrogenase, and 21-hydroxylase immunoreactivity in each adenoma. Dehydroepiandrosteronesulfotransferase immunoreactivity was pronounced in the left adenoma, less pronounced in one of the right adenoma and weak in the other right adenoma. These results were consistent with clinical endocrine findings. Ours is the first case of a patient with bilateral cortisol-secreting adenomas with unilateral oversecretion of dehydroepiandrosterone sulphate. Resumption of menstruation after the correction of the dehydroepiandrosterone-sulphate excess, despite persistent cortisol excess, indicates the importance of adrenal androgens for the regulation of the menstrual cycle.

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“…A previously described protocol was used 7 with some changes. The authors of the original protocol propose the measurement of epinephrine as a method to determine the proper location of the catheters when performing the sampling.…”

Section: Case Reportmentioning

confidence: 99%

Uso del muestreo venoso adrenal en un paciente con un síndrome de Cushing adrenal

et al. 2015

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The primary bilateral macronodular adrenal hyperplasia or the independent adrenocorticotropic hormone bilateral nodular adrenal hyperplasia is a rare cause hypercortisolism, its diagnosis is challenging and there is no clear way to decide the best therapeutic approach. Adrenal venous sampling is commonly used to distinguish the source of hormonal production in patients with primary hyperaldosteronism. It could be a useful tool in this context because it might provide information to guide the treatment. We report the case of a patient with ACTH independent Cushing syndrome in whom the use of adrenal venous sampling with some modifications radically modified the treatment and allowed the diagnosis of a macronodular adrenal hyperplasia.

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Challenges in the Diagnostic Work-Up and Management of Patients with Subclinical Cushing's Syndrome and Bilateral Adrenal Masses

Cited by 22 publications

References 22 publications

ACTH-independent Cushing’s syndrome with bilateral cortisol-secreting adrenal adenomas: a case report and review of literatures

ACTH-independent Cushing’s syndrome with bilateral cortisol-secreting adrenal adenomas: a case report and review of literatures

A case of Cushing’s syndrome due to bilateral cortisol-secreting adenomas with unilateral DHEAS oversecretion

Uso del muestreo venoso adrenal en un paciente con un síndrome de Cushing adrenal

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Challenges in the Diagnostic Work-Up and Management of Patients with Subclinical Cushing's Syndrome and Bilateral Adrenal Masses

Cited by 22 publications

References 22 publications

ACTH-independent Cushing’s syndrome with bilateral cortisol-secreting adrenal adenomas: a case report and review of literatures

ACTH-independent Cushing’s syndrome with bilateral cortisol-secreting adrenal adenomas: a case report and review of literatures

A case of Cushing&rsquo;s syndrome due to bilateral cortisol-secreting adenomas with unilateral DHEAS oversecretion

Uso del muestreo venoso adrenal en un paciente con un síndrome de Cushing adrenal

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A case of Cushing’s syndrome due to bilateral cortisol-secreting adenomas with unilateral DHEAS oversecretion