Differential Diagnosis of Adrenocorticotropic Hormone-Independent Cushing Syndrome: Role of Adrenal Venous Sampling

Martins, Raquel G.; Agrawal, Reshma; Berney, Daniel M.; Reznek, Rodney H.; Matson, Matthew; Grossman, Ashley; Druce, Maralyn

doi:10.4158/ep12136.cr

Cited by 16 publications

(18 citation statements)

References 11 publications

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“…Adrenal vein sampling has also been performed in patients with pheochromocytoma with no visible source on cross-sectional imaging (4) . Only a few studies have reported the use of adrenal venous sampling for Cushing’s syndrome with bilateral masses, of which the most well known was conducted by the Mayo Clinic (Rochester, MN, USA) ( 2 , 5 , 6 ). Young and coworkers proposed cut-off values to determine unilateral or bilateral cortisol hypersecretion based on an assessment of 10 patients.…”

Section: Discussionmentioning

confidence: 99%

A case of adrenal Cushing’s syndrome with bilateral adrenal masses

Guo

Hwu

Won

et al. 2016

Endocrinology, Diabetes &Amp; Metabolism Case Reports

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SummaryA functional lesion in corticotrophin (ACTH)-independent Cushing’s syndrome is difficult to distinguish from lesions of bilateral adrenal masses. Methods for distinguishing these lesions include adrenal venous sampling and 131I-6β-iodomethyl-19-norcholesterol (131I-NP-59) scintigraphy. We present a case of a 29-year-old Han Chinese female patient with a history of hypercholesterolaemia and polycystic ovary syndrome. She presented with a 6month history of an 8kg body weight gain and gradual rounding of the face. Serial examinations revealed loss of circadian rhythm of cortisol, elevated urinary free-cortisol level and undetectable ACTH level (<5pg/mL). No suppression was observed in both the low- and high-dose dexamethasone suppression tests. Adrenal computed tomography revealed bilateral adrenal masses. Adrenal venous sampling was performed, and the right-to-left lateralisation ratio was 14.29. The finding from adrenal scintigraphy with NP-59 was consistent with right adrenal adenoma. The patient underwent laparoscopic right adrenalectomy, and the pathology report showed adrenocortical adenoma. Her postoperative cortisol level was 3.2μg/dL, and her Cushingoid appearance improved. In sum, both adrenal venous sampling and 131I-NP-59 scintigraphy are good diagnostic methods for Cushing’s syndrome presenting with bilateral adrenal masses.Learning pointsThe clinical presentation of Cushing’ syndrome includes symptoms and signs of fat redistribution and protein-wasting features.The diagnosis of patients with ACTH-independent Cushing’s syndrome with bilateral adrenal masses is challenging for localisation of the lesion.Both adrenal venous sampling and 131I-NP-59 scintigraphy are good methods to use in these patients with Cushing’s syndrome presenting with bilateral adrenal masses.

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Section: Discussionmentioning

confidence: 99%

A case of adrenal Cushing’s syndrome with bilateral adrenal masses

Guo

Hwu

Won

et al. 2016

Endocrinology, Diabetes &Amp; Metabolism Case Reports

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“…NP-59 (iodocholesterol) scintigraphy was used to lateralize the source of cortisol, but is no longer available in many countries [53]. In the largest study in 10 patients, an adrenal/peripheral vein ratio 6.5 was consistent with cortisol hypersecretion and a ratio 2.0 was in favor of bilateral source of cortisol [54]. In the largest study in 10 patients, an adrenal/peripheral vein ratio 6.5 was consistent with cortisol hypersecretion and a ratio 2.0 was in favor of bilateral source of cortisol [54].…”

Section: Surgical Therapymentioning

confidence: 99%

Primary bilateral macronodular adrenal hyperplasia

Venanzi

Alencar

Bourdeau

et al. 2014

Current Opinion in Endocrinology, Diabetes &Amp; Obesity

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Recent findings indicate that bilateral macronodular adrenal hyperplasia is more frequently genetically determined than previously believed. Considering the role of paracrine adrenocorticotropic hormone production on cortisol secretion, the previous nomenclature of adrenocorticotropic hormone-independent macronodular adrenal hyperplasia appears inappropriate, and this disease should now be named primary bilateral macronodular adrenal hyperplasia.

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“…2015;5(3):226-228 adrenal venous sampling. Aldosterone measurement in AVS, reportedly used in case reports, could have provided important information during diagnosis, given the subtly low right adrenal cortisol and low left adrenal epinephrine levels (likely from phrenic vein dilution) but based on size and hypercortisolism bilateral adrenal resection was indicated [2,3]. Persistent hypercortisolism increases the risk of perioperative morbidity by increasing infectious complications and delayed tissue healing [4].…”

Section: Discussionmentioning

confidence: 99%

Mifepristone Utilization in Cushing’s From Large Bilateral Adrenal Adenoma Prior to Surgery

et al. 2015

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Utilization of mifepristone was described to mitigate hypercortisolism from bilateral adrenal Cushing's syndrome. A 47-year-old female on abdominal pain evaluation with CT was found to have a 5.4 × 1.7 × 4.9 cm right adrenal nodule and a 4.3 × 2.9 × 3.3 cm left adrenal nodule with calcification on CT scan with each having density of -3 Housefield units. She had hypercortisolism with testing revealing high urine free cortisol, unsuppressed cortisol on 1 mg and 8 mg overnight dexamethasone suppression testing, high 11 pm salivary cortisol × 2 along with elevated random cortisol and undetectable ACTH. Her DHEA-S, plasma metanephrine, and rostenedione, renin and aldosterone levels were normal. Adrenal venous sampling for lateralization of hypercortisolism source using dexamethasone 2 mg PO Q 6 hours × 1 day, showed adrenal vein epinephrine levels step up > 100 pg/mL, indicating successful catheterization. Each side adrenal vein to vena caval cortisol ratio > 4 was consistent with autonomous cortisol secretion and left to right adrenal cortisol ratio < 2 suggested bilateral adrenal Cushing's syndrome. She had multiple co-morbidities including uncontrolled hypertension, poor functional status requiring walker and cane, chronic non-healing cellulitis, glucose intolerance and hypokalemia. Mifepristone was initiated for medical management of hypercortisolism prior to surgery, resulting in > 50 lbs weight loss, improved blood pressure, complete healing of chronic lower extremity cellulitis and independent ambulation within 4 months. Initially right adrenal adenoma and then left adrenal adenoma were resected in two separate surgeries and pathology showed bilateral enlarged adrenal glands with multiple adrenocortical nodules without adrenal carcinoma. Mifepristone, FDA approved for inoperable Cushing's, facilitated pre-surgery optimization by effectively mitigating hypercortisolism. Mifepristone was utilized as a bridge for Cushing's medical management prior to surgery.

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Differential Diagnosis of Adrenocorticotropic Hormone-Independent Cushing Syndrome: Role of Adrenal Venous Sampling

Cited by 16 publications

References 11 publications

A case of adrenal Cushing’s syndrome with bilateral adrenal masses

A case of adrenal Cushing’s syndrome with bilateral adrenal masses

Primary bilateral macronodular adrenal hyperplasia

Mifepristone Utilization in Cushing’s From Large Bilateral Adrenal Adenoma Prior to Surgery

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