Epinephrine to the Rescue: Identifying the Offending Side in Bilateral (B/L) Macronodular Adrenal Hyperplasia (BMAH) With Cushing Syndrome (CS) and Hyperaldosteronism

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.

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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

Journal of the Endocrine Society ◽

10.1210/jendso/bvaa046.2191 ◽

2020 ◽

Vol 4 (Supplement_1) ◽

Author(s):

Yu Mi Kang ◽

Sachin Majumdar

Keyword(s):

Cushing Syndrome ◽

Urine Volume ◽

Adrenal Vein ◽

Adrenal Venous Sampling ◽

Venous Sampling ◽

Unilateral Adrenalectomy ◽

Free Cortisol ◽

Adrenal Masses ◽

The Common ◽

The Right

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.

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Unilateral Adrenalectomy for Primary Bilateral Macronodular Adrenal Hyperplasia: Analysis of 71 Cases

Experimental and Clinical Endocrinology & Diabetes ◽

10.1055/a-0998-7884 ◽

2019 ◽

Vol 128 (12) ◽

pp. 827-834

Author(s):

Mohammad Sheikh-Ahmad ◽

Gabriel Dickstein ◽

Ibrahim Matter ◽

Carmela Shechner ◽

Jacob Bejar ◽

...

Keyword(s):

Medical Center ◽

Urinary Free Cortisol ◽

Adrenal Hyperplasia ◽

Unilateral Adrenalectomy ◽

Long Period ◽

Pubmed Database ◽

Free Cortisol ◽

Adrenal Masses ◽

Current Article

Abstract Objective Primary bilateral macronodular adrenal hyperplasia (PBMAH) is characterized by benign bilateral enlarged adrenal masses, causing Cushing’s syndrome (CS). The aim of the current article is to define the role of unilateral adrenalectomy (UA) in treating patients with CS related to PBMAH. Methods A PubMed database search was conducted to identify articles reporting UA to treat PBMAH. We also report cases of PBMAH from our medical center treated by UA. Results A total number of 71 cases of PBMAH (62 cases reported in the literature and 9 cases from our center) are presented. Most patients were women (73.2%) and most UA involved the left side (64.3%). In most cases, the resected gland was the larger one. Following UA, 94.4% of cases had remission of hypercortisolism. Recurrence rate of CS was 19.4% and hypoadrenalism occurred in 29.6%. After UA, when the size of the remained adrenal gland was equal or greater than 3.5 cm, CS persisted in 21.4% of cases, and recurrence occurred in 27.3% of cases (after 20±9.2 months). However, when the size of the remained gland was less than 3.5 cm, CS resolved in all cases and recurrence occurred in 21.2% of cases after a long period (65.6±52.1 months). High levels of urinary free cortisol (UFC) were not correlated with post-surgical CS recurrence or persistence. Conclusions UA leads to beneficial outcomes in patients with CS related to PBMAH, also in cases with pre-surgical elevated UFC or contralateral large gland.

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Adrenal Venous Sampling in Primary Aldosteronism: The Usefulness of Contralateral Suppression Index

Case Reports in Medicine ◽

10.1155/2019/1604367 ◽

2019 ◽

Vol 2019 ◽

pp. 1-4 ◽

Cited By ~ 1

Author(s):

Natalia Treistman ◽

Aline Barbosa Moraes ◽

Stéphanie Cozzolino ◽

Patrícia de Fatima dos Santos Teixeira ◽

Leonardo Vieira Neto

Keyword(s):

Primary Aldosteronism ◽

Vena Cava ◽

Adrenal Vein ◽

Adrenal Venous Sampling ◽

Venous Sampling ◽

Successful Cannulation ◽

Contralateral Suppression ◽

The Right ◽

Cortisol Ratio ◽

Suppression Index

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.

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MON-LB44 Cushing’s Syndrome and Illegal Receptors

Journal of the Endocrine Society ◽

10.1210/jendso/bvaa046.2203 ◽

2020 ◽

Vol 4 (Supplement_1) ◽

Author(s):

Caroline Poku ◽

Randa Sharag Eldin ◽

Abubakr Hassab Elrasoul Babiker Mohamed ◽

Sadiq Sobia

Keyword(s):

Cushing’S Syndrome ◽

Hormone Receptors ◽

Age Distribution ◽

Cushing's Syndrome ◽

Endocrine Surgery ◽

Adrenal Hyperplasia ◽

Unilateral Adrenalectomy ◽

Production Studies ◽

Bilateral Adrenal Hyperplasia ◽

Adrenal Masses

Abstract Case presentation: A 48-year-old female with HTN presented to the endocrinology clinic for the evaluation of incidental bilateral adrenal masses noted on chest CT for dyspnea workup. At the time of the presentation, she reported generalized fatigue, significant weight gain in the past year and shortness of breath. Her physical exam was remarkable for central obesity. Lab work showed elevated cortisol after 1 mg dexamethasone suppression test x 2 and elevated 24- hour urine cortisol. Plasma free metanephrine levels and aldosterone/ renin ratio were normal. MRI abdomen was done and showed bilateral adrenal masses (left: 5.6 cm, right: 3.2 cm). Patient was diagnosed with Cushing’s syndrome secondary to primary bilateral adrenal hyperplasia and was referred to endocrine surgery who recommended unilateral adrenalectomy. The decision was made to remove the larger left side adrenal mass. On post-operative day one her am cortisol decreased to 2.1 and she was started on hydrocortisone 20 mg in the morning and 10 mg in the evening. Discussion: Primary bilateral adrenal hyperplasia is a rare cause (< 2 %) of endogenous Cushing’s syndrome, usually occurs in a bimodal age distribution, in childhood and in the fifth- sixth decades. Presentation is variable with most patients having no symptoms or subclinical Cushing’s. The theory is the larger nodule size corelates with the higher cortisol production. Studies have shown between 60-70% of cases has aberrant ectopic hormone receptors which leads to increased cortisol production not only from ACTH but also from other ligands such as serotonin and vasopressin. Aberrant receptor testing examines whether cortisol or other steroid production increases in response to either physiologic or pharmacologic stimulus. Multiple genetic mutations have been associated, the most frequent is mutations in the Armadillo repeat- containing 5 gene identified in 2013. Treatment can either be medical or surgical. Medical therapy can be initiated if testing for an aberrant receptor is positive. In recent years there has been a trend towards doing unilateral adrenalectomy instead of bilateral, with initial remission of symptoms reported in about 84% of cases after unilateral adrenalectomy although there is a small risk of recurrence. Post operatively after unilateral adrenalectomy patients should be monitored for adrenal insufficiency. Our patient declined aberrant receptor testing and opted for surgery and is doing well post operatively. Conclusion: Primary bilateral adrenal hyperplasia is a rare cause of endogenous Cushing’s syndrome which can be treated either medically or surgically.

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Stereotactic radiosurgery for Cushing disease

Neurosurgical FOCUS ◽

10.3171/foc.2004.16.4.6 ◽

2004 ◽

Vol 16 (4) ◽

pp. 1-7 ◽

Cited By ~ 12

Author(s):

Stephen J. Hentschel ◽

Ian E. McCutcheon

Keyword(s):

Stereotactic Radiosurgery ◽

Cortisol Level ◽

Cushing Syndrome ◽

Latency Period ◽

Serum Cortisol ◽

Serum Cortisol Level ◽

Cushing Disease ◽

Fractionated Radiotherapy ◽

Free Cortisol ◽

Microsurgical Resection

The most common cause of Cushing syndrome is Cushing disease, in which hypercortisolism is produced by a functional adrenocorticotropic hormone–producing adenoma of the anterior pituitary gland. The common therapies available include microsurgical resection, conventional fractionated radiotherapy, and stereotactic radiosurgery (SRS). In this article the authors review the indications, results, and complications associated with SRS in the treatment of Cushing disease. In as many as 90% of patients SRS results in disease remission, which is defined as a normal 24-hour urinary free cortisol level and a normal or subnormal morning serum cortisol level. Although in most patients who are subsequently cured a marked decrease in the serum cortisol level is demonstrated within 3 months after treatment, a biochemical cure may be delayed up to 3 years in some cases. Complications following SRS for pituitary adenomas are uncommon, particularly in patients with microadenomas, which are most commonly seen in Cushing disease. The most common complication is hypopituitarism, which occurs in up to 50% of patients with a mean latency period of 5 years. Radiation-induced optic neuropathy has been reported in less than 2% of cases and induction of a secondary neoplasm in less than 1% of cases. For patients with Cushing disease, the rate of endocrinological cure following SRS appears to be similar to that attained using microsurgical resection. In contrast to surgery, SRS has the benefit of being noninvasive and associated with a very low incidence of diabetes insipidus, although hypopituitarism may be more common with SRS. With continued follow-up patient reviews and additional experience with SRS, it may become possible to make more definitive statements regarding SRS as the initial treatment for patients with Cushing disease.

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

Acta Endocrinologica ◽

10.1530/eje.0.1460237 ◽

2002 ◽

pp. 237-240 ◽

Cited By ~ 41

Author(s):

C Lamas ◽

JJ Alfaro ◽

T Lucas ◽

B Lecumberri ◽

B Barcelo ◽

...

Keyword(s):

Adrenal Insufficiency ◽

Replacement Therapy ◽

Cushing’S Syndrome ◽

Alternative Treatment ◽

Cushing's Syndrome ◽

Adrenal Hyperplasia ◽

Unilateral Adrenalectomy ◽

Free Cortisol

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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Diagnosis and management of primary bilateral macronodular adrenal hyperplasia

Endocrine Related Cancer ◽

10.1530/erc-19-0240 ◽

2019 ◽

Vol 26 (10) ◽

pp. R567-R581 ◽

Cited By ~ 4

Author(s):

Dimitra A Vassiliadi ◽

Stylianos Tsagarakis

Keyword(s):

Genetic Defect ◽

Urinary Free Cortisol ◽

Distinct Pattern ◽

Cortisol Secretion ◽

Adrenal Hyperplasia ◽

Unilateral Adrenalectomy ◽

Steroid Dependency ◽

Downstream Signaling ◽

Free Cortisol ◽

Hormonal Evaluation

Primary bilateral macronodular adrenal hyperplasia (PBMAH) is a highly heterogeneous entity. The incidental identification of an increasing number of cases has shifted its clinical expression from the rarely encountered severe forms, regarding both cortisol excess and adrenal enlargement, to mild forms of asymptomatic or oligosymptomatic cases with less impressive imaging phenotypes. Activation of cAMP/PKA pathway, either due to alterations of the different downstream signaling pathways or through aberrantly expressed G-protein-coupled receptors, relates to both cortisol secretion and adrenal growth. Germline ARMC5 mutations are a frequent genetic defect. The diagnostic approach consists of both imaging and hormonal characterization. Imaging characterization should be done separately for each lesion. Endocrine evaluation in cases with clinically overt Cushing’s syndrome (CS) is similar to that applied for all forms of CS. In incidentally detected PBMAH, hormonal evaluation includes testing for primary aldosteronism, pheochromocytoma and evaluation for autonomous cortisol secretion, using the 1 mg overnight dexamethasone suppression test. Midnight cortisol or 24-h urinary free cortisol may aid in establishing the degree of cortisol excess. In patients with hypercortisolism, ACTH levels should be measured in order to establish ACTH independency. At variance with other forms of CS, PBMAH may be characterized by a distinct pattern of inefficient steroidogenesis. The appropriate management of PBMAH remains controversial. Bilateral adrenalectomy results in lifetime steroid dependency and is better reserved only for patients with severe CS. Unilateral adrenalectomy might be considered in selected patients. In cases where the regulation of cortisol secretion is mediated by aberrant receptors there is some potential for medical therapy.

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Giant Bilateral Adrenal Myelolipoma with Congenital Adrenal Hyperplasia

Case Reports in Surgery ◽

10.1155/2014/728198 ◽

2014 ◽

Vol 2014 ◽

pp. 1-5 ◽

Cited By ~ 4

Author(s):

S. Al-Bahri ◽

A. Tariq ◽

B. Lowentritt ◽

D. V. Nasrallah

Keyword(s):

Congenital Adrenal Hyperplasia ◽

Abdominal Distension ◽

Adrenal Hyperplasia ◽

Hematopoietic Tissue ◽

Hydroxylase Deficiency ◽

Adrenal Myelolipoma ◽

Massive Growth ◽

History Of ◽

Adrenal Masses ◽

The Right

Myelolipomas are rare and benign neoplasms, predominant of the adrenal glands, consisting of adipose and mature hematopoietic tissue, commonly discovered incidentally with increased use of radiologic imaging. Few cases of giant bilateral adrenal masses are reported, especially in the setting of congenital adrenal hyperplasia (CAH). We report the case of a 39-year-old male with a history of CAH secondary to 21-αhydroxylase deficiency on steroids since childhood, self-discontinued during adolescence, presenting with abdominal distension, fatigue, decreased libido, and easy bruising. Imaging revealed giant bilateral adrenal masses. He subsequently underwent bilateral adrenalectomy found to be myelolipomas measuring 30 × 25 × 20 cm on the left and weighing 4.1 kg and 25 × 20 × 13 cm on the right and weighing 2.7 kg. Adrenal myelolipomas are found to coexist with many other conditions such as Cushing’s syndrome, Addison’s disease, and CAH. We discuss the association with high adrenocorticotropic hormone (ACTH) states and review the studies involving ACTH as proponent leading to myelolipomas. Massive growth of these tumors, as in our case, can produce compression and hemorrhagic symptoms. We believe it is possible that self-discontinuation of steroids, in the setting of CAH, may have resulted in the growth of his adrenal masses.

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MON-193 Glucocorticoid Excess and Its Association with Metabolic and Cardiovascular Complications in Primary Aldosteronism

Journal of the Endocrine Society ◽

10.1210/jendso/bvaa046.1758 ◽

2020 ◽

Vol 4 (Supplement_1) ◽

Author(s):

Ke ying zhu ◽

huan chen ◽

Liu mengsi ◽

Wen-huan huan Feng ◽

da long zhu ◽

...

Keyword(s):

Cortisol Level ◽

Primary Aldosteronism ◽

Linear Regression Analysis ◽

Laboratory Data ◽

Cortisol Concentration ◽

Serum Cortisol Level ◽

Cortisol Secretion ◽

Increased Risk ◽

Free Cortisol ◽

Midnight Cortisol

Abstract Objective: To investigate autonomous cortisol secretion in patients with primary aldosteronism and its effect on metabolism and cardiovascular events (CVE) in patients with primary aldosteronism (PA). Methods: This study included 163 patients with PA and 105 sex- and age-matched patients with essential hypertension (EH). Clinical and laboratory data were collected. The expression of cortisol synthase (CYP11B1) and aldosterone synthase (CYP11B2) was investigated in adenoma tissues from 44 patients with aldosterone-producing adenoma (APA) by employing immunohistochemistry. Results: 1) CYP11B2 was expressed in 36 patients with APA and was absent in 8 patients, while CYP11B1 immunoreactivity was detected in all tested patients with APA; 2) Compared with patients with EH, midnight cortisol concentration (104.81±90.86 vs 76.87±65.86), 24-h urine free cortisol level (726.04±309.87 vs 630.65±168.2), and cortisol level after 1mg-DST [35.6(27.6,75.73)vs 27.6(27.60,29.27)]were all significantly increased in patients with PA(all P<0.01); 3) Midnight cortisol concentration is positively associated with systolic blood pressure (SBP) (r=0.147 P<0.05), LDL-C (r=0.194 P<0.05), HOMA-IR (r=0.262 P<0.05), HOMA-β (r=0.313 P<0.05) and fasting insulin level (r=0.329 P<0.05) in patients with PA. In multiple linear regression analysis, midnight cortisol concentration was a postitive predictor for SBP (β=0.185 P<0.05), HOMA-IR (β=0.331 P<0.05) and HOMA-β (β=0.390 P<0.05); 4) Of the 163 patients with PA, 35 had a history of CVE, including 22 with stroke, eight with myocardial infarction, three with atrial fibrillation, and two with heart failure. The multivariate logistic-regression analyses revealed older age (OR 1.160 95%CI 1.041-1.294 P <0.001) and higher serum aldosterone level (OR 1.013 95%CI 1.000-1.026 P <0.05) were independent risk factors of CVE, serum cortisol level was not associated with the increased risk of CVE in patients with PA (OR 0.997 95%CI 0.985-1.009 P =0.570). Conclusion: Excess cortisol secretion was common in PA. Cortisol excretion is correlated with metabolic disorders, but not associated with increased risk of CVE in patients with PA.

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Cushing’s Disease (CD) Due to ACTH-Secreting Pituitary Microadenoma Incidentally Discovered on a Sestamibi Scan for Primary Hyperparathyroidism

Journal of the Endocrine Society ◽

10.1210/jendso/bvab048.1182 ◽

2021 ◽

Vol 5 (Supplement_1) ◽

pp. A579-A580

Author(s):

Hassan Mehmood ◽

Murray B Gordon

Keyword(s):

Pituitary Adenoma ◽

Primary Hyperparathyroidism ◽

Pituitary Adenomas ◽

Case Reports ◽

Adrenal Hyperplasia ◽

Pituitary Microadenoma ◽

Free Cortisol ◽

Sestamibi Scan ◽

The Right ◽

Acth Secreting

Abstract Introduction: MIBI scintigraphy is commonly being used for the preoperative localization of parathyroid adenomas. Multiple studies showed MIBI uptake in pituitary adenomas are likely due to higher metabolic activity. When hyperfunctioning pituitary adenomas were reported, both had CD [1,2]. We present the third case of increased pituitary uptake on a MIBI scan later confirmed as CD. CaseA 64-year-old Caucasian female s/p renal transplantation for RPGN who presented for evaluation of hypercalcemia. Evaluation confirmed primary hyperparathyroidism with persistently elevated PTH levels 74-108 pg/ml (11-68), serum calcium levels 10.0-10.4 mg/dl (8.4-10.3), albumin 4.1-4.3 g/dl (3.6-5.1), phosphorus 3.0-3.2 mg/dl (2.5-4.5), creatinine 0.94-1.07 mg/dl. 24-hour urine calcium 60 mg/day (35-250). Vitamin D-25 OH level was 37 ng/dL (30-100). A sestamibi scan showed uptake in the right lower parathyroid, the midsternal chest region and the pituitary gland. MRI of the pituitary revealed a 7mm cystic pituitary microadenoma in the right posterior pituitary. CD was confirmed by the findings of persistently elevated 8 AM serum cortisol levels of 28.4 and 24.2 mcg/dl (4-22), ACTH levels of 59 and 39 pg/ml (10-48), and an elevated plasma free cortisol of 1.43 mcg/dl (0.07-0.93). CT of the abdomen showed L adrenal thickening suggesting adrenal hyperplasia from CD. Plasma cortisol suppressed to 1.2 mg/dl following 1 mg of dexamethasone. 24 urine for free cortisol 26.7 mcg/day (4-50). The patient had no proximal muscle weakness, striae or Cushingoid facial features. She had no hyperglycemia or hypertension. Patient was diagnosed with an ACTH secreting pituitary microadenoma with mild CD and adrenal hyperplasia. Her DXA scan showed osteoporosis. Genetic testing for MEN1 mutation was negative. Patient did not wish surgery for either her hyperparathyroidism or her CD and is being evaluated for medical treatment of hypercortisolism. Conclusion: There are two prior case reports of an incidentally discovered pituitary adenoma on sestamibi scan later diagnosed as CD [1,2]. Corticotrophs may have a strong affinity for sestamibi. Our case is the first, to our knowledge, of pituitary MRI confirmation of the ACTH secreting pituitary incidentaloma initially suspected by pituitary uptake on a sestamibi scan in a patient with hyperparathyroidism. Reference1. Kuhadiya ND et al. Incidentally Discovered ACTH-Secreting Pituitary Adenoma on a Sestamibi Scan in a Patient With Hyperparathyroidism. AACE Clinical Case Reports. 2015;1(3):e152-5. 2. Gierach M et al. The case of Cushing’s disease imaging by SPECT examination without manifestation of pituitary adenoma in MRI examination. Nuclear Medicine Review. 2005;8(2):137-9.

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