scholarly journals MON-LB035 Adrenal Venous Sampling in the Lateralization of Acth-Independent Cushing Syndrome With Bilateral Adrenal Masses: A Case With a 5-Year Follow-Up

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 5 (Supplement_1) ◽  
pp. A130-A131
Author(s):  
Tarunya Vedere ◽  
Parvathy Madhavan ◽  
Carl D Malchoff

Abstract Management of ACTH-independent CS in the setting of B/L adrenal masses can be challenging. We present a patient with concomitant cortisol and aldosterone hypersecretion and B/L adrenal masses who was treated with unilateral adrenalectomy (UA) based on simultaneous B/L adrenal vein sampling (AVS) results normalized to plasma epinephrine levels, to correct for flow and dilution. A 60-year-old lady presented for evaluation of incidentally discovered B/L adrenal masses. CT abdomen with contrast revealed B/L lobulated adrenal glands measuring 3.4 x 5.5 x 4.1 cm on the right and 3.8 x 4.2 x 2 cm on the left (pre contrast density of 37 and 44 Hounsfield Units respectively). She had a recent diagnosis of HTN and prediabetes. She reported fatigue, easy bruising, and muscle weakness. Family history was notable for a sister with CS and B/L adrenal masses who was successfully treated with UA. On physical examination her BP was 160/94 mm Hg and she did not have an obvious cushingoid appearance. Laboratory testing included an elevated AM serum cortisol level of 16.6 mcg/dL following 1 mg of dexamethasone given at 11 pm the previous night. ACTH was <1.0 pg/mL consistent with ACTH-independent CS. She had a serum aldosterone level of 26.6 ng/dL(4.0 - 31.0 ng/dL),simultaneous plasma renin activity of 0.5 ng/ml/hr (0.5–4 ng/ml/hr) and serum potassium level of 4.4 mmol/l. She underwent B/L simultaneous AVS. Given concomitant hyperaldosteronism, plasma epinephrine levels were measured to determine successful catheter placement and to correct for flow and dilution between the adrenal veins. Right adrenal vein to peripheral vein cortisol ratio was 9.27 (> 6.5) consistent with cortisol hypersecretion. The right to left cortisol ratio was 2.3 (>/= 2.3), consistent with right sided predominance. Right to left ratio of the epinephrine corrected cortisol level was 2.7. She underwent right adrenalectomy, and pathology showed macronodular adrenal hyperplasia. Postoperatively, she had resolution of HTN and normalization of 24 hour urine free cortisol. UA is increasingly becoming recognized as a successful treatment for BMAH due to lower risk of adrenal crisis when compared to B/L adrenalectomy. In a recent publication, remission from CS was seen in 94.4 % of patients with BMAH following UA, with recurrence in 19% of patients. The mean time to recurrence was 5 years. We utilized cutoff values that were previously reported for cortisol lateralization in interpreting AVS. Our patient attained remission of CS 9 months post UA. Epinephrine levels were not significantly different between the adrenal veins, possibly due to simultaneous AVS, so we were also able to calculate epinephrine corrected cortisol ratios. Simultaneous B/L AVS with epinephrine to localize autonomous hypercortisolism in the setting of concomitant hyperaldosteronism can be beneficial in guiding surgical management of patients.


2019 ◽  
Vol 2019 ◽  
pp. 1-4 ◽  
Author(s):  
Natalia Treistman ◽  
Aline Barbosa Moraes ◽  
Stéphanie Cozzolino ◽  
Patrícia de Fatima dos Santos Teixeira ◽  
Leonardo Vieira Neto

Adrenal venous sampling (AVS) is the gold standard test to differentiate the unilateral from the bilateral form in patients with primary aldosteronism (PA) although it may be a difficult procedure, especially the successful cannulation of the right adrenal vein. In this report, we describe a 49-year-old female patient diagnosed with PA, after investigating resistant hypertension and refractory hypokalemia. Abdominal computed tomography scan revealed a 2.5 cm adenoma on the right adrenal vein. AVS was performed under cosyntropin infusion. Aldosterone and cortisol concentrations were obtained from the right and left adrenal veins and inferior vena cava (IVC). Cortisol on each adrenal vein divided by cortisol on IVC confirmed successful cannulation of the left side only, which makes it impossible to calculate the lateralization index (LI). From the data on the left adrenal vein and IVC, the aldosterone-to-cortisol ratio divided by the IVC aldosterone-to-cortisol ratio was less than 1.0, suggesting that the left adrenal vein was suppressed with the excess aldosterone originating from the contralateral side (contralateral suppression index (CSI)). Right adrenalectomy was performed; postoperative hypoaldosteronism was confirmed. This report highlights the importance of CSI obtained in AVS when technical difficulties occur making it impossible to obtain LI, which is most commonly used to decide between surgical and clinical management of PA.


2015 ◽  
Vol 26 (6) ◽  
pp. 910-914 ◽  
Author(s):  
Sota Oguro ◽  
Seishi Nakatsuka ◽  
Hideki Yashiro ◽  
Subaru Hashimoto ◽  
Kazutoshi Miyashita ◽  
...  

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.


Surgery ◽  
2016 ◽  
Vol 159 (1) ◽  
pp. 267-274 ◽  
Author(s):  
Jesse D. Pasternak ◽  
Irene Epelboym ◽  
Natalie Seiser ◽  
Matt Wingo ◽  
Max Herman ◽  
...  

2013 ◽  
Vol 24 (4) ◽  
pp. 550-557 ◽  
Author(s):  
Akira Takada ◽  
Kojiro Suzuki ◽  
Yoshine Mori ◽  
Ryota Hyodo ◽  
Kenichi Kawakami ◽  
...  

2019 ◽  
Vol 2019 ◽  
pp. 1-6
Author(s):  
Wenjing Zhang ◽  
Keying Zhu ◽  
Hongyun Li ◽  
Yan Zhang ◽  
Dalong Zhu ◽  
...  

The management of patients with adrenocorticotropic hormone-independent Cushing’s syndrome and bilateral adrenal masses is challenging. Adrenal venous sampling (AVS) has been used to identify functional lesions in previous studies, but it is not always reliable. The present study aims to address the variability of cortisol in the adrenal veins of patients without excessive cortisol secretion and investigate the use of adrenal androgens to correct the cortisol lateralization ratio in AVS. Thirty-seven patients with primary aldosteronism underwent successful AVS. Patients with normal cortisol secretion exhibited a wide range of cortisol concentrations in the right (601-89, 400 nmol/l) and left (331-35, 300 nmol/l) adrenal veins. The median cortisol gradients between adrenal venous and peripheral venous samples were 15.25 and 10.14 in the right and left sides, respectively, and the cortisol lateralization ratio (high side to low side) was as high as 9.49 (median 1.54). The mean plasma levels of cortisol in the adrenal venous and peripheral venous samples decreased from t-15 to t0. Significant positive correlations were observed between the cortisol concentrations and both androstenedione and dehydroepiandrosterone concentrations in the right and left adrenal veins. After correcting for androstenedione or dehydroepiandrosterone levels, the cortisol lateralization ratio was less than 2 in most adrenal venous samples. The present study demonstrated the wide variation in cortisol concentrations in the adrenal veins of patients with normal cortisol secretion. The adrenal androgens might be ideal analytes used as normalizers when assessing the cortisol lateralization of AVS in normal or hypercortisolism cases.


2021 ◽  
pp. 028418512110340
Author(s):  
Soma Kumasaka ◽  
Hiroyuki Tokue ◽  
Yoshito Tsushima

Background Primary aldosteronism is one of the most common causes of secondary hypertension. Unilateral primary aldosteronism can be treated with adrenalectomy; therefore, determining laterality is essential, for which adrenal venous sampling is considered the gold standard. However, as catheter insertion and sampling at an appropriate venous point is occasionally difficult, it is a time-consuming procedure. Purpose To evaluate the patient characteristics and imaging findings that influence the adrenal venous sampling procedure. Material and Methods A total of 69 patients who underwent adrenal venous sampling between January 2013 and December 2017 were retrospectively analyzed. The procedure was considered difficult if the duration was > 142 min (mean ± standard deviation [SD] of procedure time in this study) and/or proper sampling failed. Anatomical factors such as belly diameter, presence of adrenal nodules, diameter of the right adrenal vein and inferior vena cava, ratio of the diameters of the right adrenal vein to diameter of the inferior vena cava, vertical direction of the right adrenal vein, and vertebral level of the right adrenal vein were evaluated as predictive factors on computed tomography. Results Fifteen patients (21.7%) were considered difficult cases. The factors associated with difficulty were the long transverse diameter of the belly ( P = 0.004) and high vertebral level of the right adrenal vein ( P = 0.032). No statistical differences were observed in any other factors. Conclusion The long transverse diameter of the belly and high vertebral level of the right adrenal vein may prevent completion of the adrenal venous sampling procedure.


Sign in / Sign up

Export Citation Format

Share Document