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

2012 ◽  
Vol 18 (6) ◽  
pp. e153-e157 ◽  
Author(s):  
Raquel Martins ◽  
Reshma Agrawal ◽  
Daniel Berney ◽  
Rodney Reznek ◽  
Matthew Matson ◽  
...  
2017 ◽  
Vol 103 (3) ◽  
pp. 803-808
Author(s):  
Shi Chen ◽  
Ran Li ◽  
Xiaobo Zhang ◽  
Lin Lu ◽  
Ji Li ◽  
...  

Abstract Context Cushing syndrome is rarely caused by the secretion of cortisol from ovarian tumors. In clinical decision-making, it is important to determine whether the ovarian tumor is capable of secreting cortisol. Selective ovarian and adrenal venous sampling is scarcely reported in the localization of ACTH-independent ectopic Cushing syndrome. Case Description We present a case of 40-year-old Chinese woman who had weight gain, hirsutism, hypertension, and menstrual disorder over 6 months. Her physical examination and biochemical assessment revealed adrenocorticotropic hormone-independent Cushing syndrome. Adrenal computed tomography scan indicated no abnormality. A mass of 5.7 cm × 4.2 cm × 3.4 cm was discovered by pelvic ultrasonography. Somatostatin receptor scintigraphy revealed no abnormal radioactivity intake. Combined ovarian and adrenal venous sampling together with a cortisol assay were conducted. Results revealed cortisol concentration of the right-side ovarian vein, left-side ovarian vein, and peripheral vein of 268.60, 29.00, and 35.18 μg/dL, respectively, suggesting a right-side ovarian origin. A right-side salpingo-oophorectomy was performed and the pathological diagnosis revealed ovarian steroid cell tumor, not otherwise specified. The cortisol level was substantially lower after the patient underwent surgery and symptoms of Cushing syndrome disappeared. At 3-year follow-up, the patient remained disease free, and no tumor was observed on pelvic ultrasonogram. Conclusion Combined ovarian and adrenal venous sampling is valuable in the localization of adrenocorticotropic hormone–independent ectopic Cushing syndrome.


2015 ◽  
pp. 84-87 ◽  
Author(s):  
Carlos Esteban Builes Montaño ◽  
Carlos Andres Villa Franco ◽  
Alejandro Román Gonzalez ◽  
Alejandro Vélez Hoyos ◽  
Santiago Echeverri Isaza

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.


2018 ◽  
Author(s):  
Laura Delegido-Gomez ◽  
Raquel Miralles-Moragrega ◽  
Clara Navarro-Hoyas ◽  
Victoria Gonzalez ◽  
Fernando Sanchez-Blanco ◽  
...  

2020 ◽  
Vol 9 (4) ◽  
Author(s):  
Dathe Z Benissan-Messan ◽  
Robert E Merritt ◽  
Konstantin Shilo ◽  
Desmond M D'Souza ◽  
Peter J Kneuertz

Ectopic adrenocorticotropic hormone ( ACTH) syndrome is rare and identification of its source is often challenging. We report the case of an ectopic Cushing syndrome in a young adult male secondary to an occult ACTH producing atypical carcinoid tumor. Extensive biochemical and imaging workup was unrevealing. The diagnosis was aided by Ga-DOTA PET scan demonstrating a suspicious left upper lobe lung nodule. The patient underwent video-assisted thoracoscopic exploration with wedge resection and mediastinal lymphadenectomy of a T2aN2M0 atypical carcinoid, resulting in the normalization of ACTH levels and complete resolution of symptoms. The role of a Ga-DOTA PET scan in diagnosing pulmonary carcinoid tumors and their management are discussed.


2019 ◽  
Vol 26 (2) ◽  
pp. 139-141 ◽  
Author(s):  
Gian Paolo Rossi ◽  
Silvia Lerco ◽  
Diego Miotto ◽  
Giulio Barbiero ◽  
Michele Battistel

2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Yu Mi Kang ◽  
Sachin Majumdar

Abstract Background Nearly 25% of adrenal Cushing syndrome (CS) patients with bilateral adrenal masses have unilateral hypercortisolism, making localization crucial for surgical planning. Since there is no standardized protocol for adrenal venous sampling (AVS) in lateralizing adrenal hypercortisolism, we share our experience with a case of CS with bilateral adrenal masses in which lateralization via AVS permitted unilateral adrenalectomy. Clinical Case A 59-year-old woman with hypertension, hyperlipidemia, and prediabetes was hospitalized for worsening back pain and hypertension. Her BMI was 26.5 kg/m2, BP 173/93 mmHg, HR 73/min, she was anxious, diaphoretic, and hirsute. Glucose was 118 mg/dL and HbA1c 6.6%. Abdominal computed tomography revealed a type B aortic dissection with both right (6.1 x 3.1cm and 3.6 x 2.4cm), and left (largest 1.7 cm) sided adrenal masses. Plasma and 24 hour-urine metanephrine, normetanephrine and catecholamines, as well as plasma renin and aldosterone levels, were normal. AM cortisol on three different occasions was 21.30, 20.70, and 21.30 mcg/dL. Midnight cortisol was 17.8 mcg/dL, and 24-hour urine free cortisol on two occasions was 163 mcg (urine volume 3.4L with creatinine 1.14) and 99.2 mcg (urine volume 1.15L). After 1mg dexamethasone her AM ACTH and cortisol were <5 and 18.70 mcg/dL, respectively. Preoperative AVS was performed and 8mg of dexamethasone was administered the night prior to ensure ACTH suppression during the procedure, and epinephrine was measured to ascertain adequate adrenal vein cannulation. Cortisol levels (in mcg/dL) from the common iliac, right and left adrenal veins were 14.7, 61.5, and 23.5 at 0 minute and 15.2, 61.0, and 22.7 at 2 minutes, respectively. Epinephrine levels (in pg/dL) from the common iliac, right and left adrenal veins were 42, 577, and 3225 at 0 minutes, and 46, 718, and 2989 at 2 minutes. Despite higher epinephrine levels from the left adrenal, the cortisol ratio of the right adrenal vein to peripheral vein was 4.18 with the right-to-left ratio of 2.59 and 2.68 at 0 and 2 minutes, suggesting hypersecretion of cortisol from the right adrenal gland. Unilateral right adrenalectomy revealed a 5.6 cm adrenal adenoma arising in a background of adrenal cortical hyperplasia. Morning postoperative cortisol was 2.2 mcg/dL. She was placed on hydrocortisone and tapered over a 10-month period with remission maintained for more than 3.5 years post-operatively. Conclusion This case demonstrates the safety, usefulness, and necessity, of AVS in localizing cortisol production when bilateral adrenal masses are present. In addition, this case suggests that the use of high dose dexamethasone and measurement of catecholamines may be helpful for more accurate interpretation. More data on AVS in CS patients with bilateral adrenal masses is needed so a well-validated and standardized CS-specific ACS protocol can be developed.


2021 ◽  
Vol 22 (1) ◽  
Author(s):  
Raluca Tulin ◽  
Adrian Tulin ◽  
Luminita Tomescu ◽  
Enyedi Mihaly ◽  
Adelaida Avino ◽  
...  

2018 ◽  
Author(s):  
Anastasia Rybakova ◽  
Ivan Sitkin ◽  
Galina Kolesnikova ◽  
Nadezda Platonova ◽  
Ekaterina Troshina ◽  
...  

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