scholarly journals Incidental Pheochromocytoma: Silent but Violent

2021 ◽  
Vol 5 (Supplement_1) ◽  
pp. A137-A138
Author(s):  
Sravani Bantu ◽  
Shirisha R Vallepu ◽  
Mouna Gunda ◽  
Vaishali Thudi
Keyword(s):  
Blood Pressure ◽  
Heart Rate ◽  
Adrenal Mass ◽  
Clinical Presentation ◽  
False Negative ◽  
Adrenal Incidentaloma ◽  
Adrenal Incidentalomas ◽  
Tumor Patient ◽  
Laboratory Errors ◽  
Pedal Edema

Abstract Background: Pheochromocytoma is a rare catecholamine secreting neuroendocrine tumor. It arises from the chromaffin cells of adrenal medulla. It is diagnosed in 5–6.5% of adrenal incidentalomas which is not common. The usual clinical presentation includes the classic triad of sweating, headache and tachycardia. However, asymptomatic cases are seen in 8% of the patients with pheochromocytoma. We present a clinically asymptomatic patient diagnosed during work up of adrenal incidentaloma. The possible etiology for silent presentation includes one of the following:(i) Presence of a smaller functional tissue (ii)Accelerated turnover of the tumor causing release of the unmetabolized catecholamines in small amounts (iii) Pulsatile tumor secretion (iv)Tumors triggered by stress (v) Laboratory errors due to inappropriate handling of specimen at high-temperature (vi) False negative test results secondary to caffeine ingestion in the prior 24 hours. Clinical Case: 59 years old Caucasian female with past medical history of type 2 diabetes mellitus, obesity, essential hypertension, nonischemic cardiomyopathy, and asthma presented to the emergency room with complaints of worsening shortness of breath and pedal edema for 1 month. Physical exam: Blood pressure 146/78 mm of Hg and heart rate 82 beats/min, mild pedal edema, no pulmonary crackles. On imaging, CT angio chest showed irregularly enhancing right adrenal mass measuring 3.4 cm. This adrenal incidentaloma was not visualized on imaging done 5 years ago. Further, MRI abdomen revealed 4.1 cm right adrenal mass. Laboratory testing showed high total plasma metanephrines: 890 pg/ml (< or = 205), 24-hour urine metanephrines: 2337 (140–785), A1C: 10%. This confirmed the diagnosis of adrenal pheochromocytoma. Preoperatively, she was started on phenoxybenzamine 10 mg BID and encouraged on liberal salt intake. During the course, her blood pressure and heart rate were monitored daily. She underwent right adrenalectomy. Surgical pathology revealed 4.1 cm pheochromocytoma, negative margins with extension to the adipose tissues and vascular invasion, PASS score = 4. Post operatively, patient declined to get labs done. Due to high risk behavior of the tumor, patient needs to be monitored annually for lifelong. Conclusion: Pheochromocytoma is an uncommon tumor with varied clinical presentation. It can manifest itself widely from being silent to aggressive disease. This warrants high suspicion, early detection and management, thereby reducing the morbidity and mortality. Lately, there has been increased incidence of adrenal incidentalomas owing to widespread use of radiological investigations. We report a case of incidental pheochromocytoma which is biochemically active but clinically asymptomatic. This emphasizes the importance of being more vigilant during the evaluation of adrenal incidentalomas.

scholarly journals SUN-154 Resistant Hypertension After Shockwave Lithotripsy: The Rude Awakening of an Adrenal Incidentaloma

2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Ramon B Larrazabal
Keyword(s):  
Blood Pressure ◽  
Adrenal Mass ◽  
Adrenal Incidentaloma ◽  
Adrenal Tumors ◽  
Shockwave Lithotripsy ◽  
Adrenocortical Adenoma ◽  
Institutional Perspective ◽  
Surgical Interventions ◽  
First Case ◽  
Free Cortisol

Abstract BACKGROUND The National Institutes of Health defines adrenal incidentalomas (AIs) as clinically inapparent adrenal masses discovered inadvertently in diagnostic testing or treatment for conditions not related to the adrenal glands. Non-functional adenoma remains the most frequent (60–85%) cause, while functional adenomas at 5–16%. CLINICAL CASE A previously healthy 41 year-old female consulted for a 2-month history of left flank pain. Computed tomography (CT) scan of the kidneys revealed left calculi and incidental right adrenal mass. She was asymptomatic at this time. She then underwent shockwave lithotripsy for the renal calculi. However, post-operatively, she had elevated blood pressure and was started on anti-hypertensives. She remained hypertensive despite being on four different medications. Pertinent physical examination findings: plethora of the face and extremities, Moon facies, Buffalo Hump, and pendulous abdomen with grayish striae. Further work-up revealed the AI to be cortisol-secreting. Pertinent laboratories: 1 mg dexamethasone suppression test - 800 nmol/L (<50 nmol/L), 24-hr urine free cortisol - 1014.86 ug/24 hr (20–90 ug/24 hr), Adrenocorticotropic Hormone (ACTH) - 5.0 pg/ml (9–52 pg/ml). She then underwent a right laparoscopic adrenalectomy. Post-operatively, her blood pressure normalized without her anti-hypertensive medications. Tissue biopsy of the mass was consistent with an adrenocortical adenoma. On her follow-up after one month, signs of Cushing’s Syndrome were clinically improving. CONCLUSION This is the first case that demonstrated shockwave lithotripsy converting a non-functioning adrenal incidentaloma into a functioning one. It also shows how internists and surgeons (i.e. Urologists) can manage various aspects of patient care through the facilitation of medical treatments, surgical interventions, and ensuring a proper multidisciplinary approach based on the endocrinology clinical guidelines. So as not to delay the delivery of proper management to the patient. REFERENCES (1) Grumbach M, Biller B, Braunstein G. et al. “Management of the clinically inapparent adrenal mass (“incidentaloma”).” Annals of internal medicine 2003; 138:424–4292 Herrera M, Grant C, van Heerden J. et al. “Incidentally discovered adrenal tumors: an institutional perspective.” Surgery 1991; 110:1014.

scholarly journals Management of adrenal incidentalomas: European Society of Endocrinology Clinical Practice Guideline in collaboration with the European Network for the Study of Adrenal Tumors

2016 ◽  
Vol 175 (2) ◽  
pp. G1-G34 ◽  
Author(s):  
Martin Fassnacht ◽  
Wiebke Arlt ◽  
Irina Bancos ◽  
Henning Dralle ◽  
John Newell-Price ◽  
...  
Keyword(s):  
Surgical Treatment ◽  
Adrenal Mass ◽  
Adrenal Incidentaloma ◽  
Serum Cortisol ◽  
Cushing's Syndrome ◽  
Cortisol Secretion ◽  
Adrenal Incidentalomas ◽  
Adrenal Masses ◽  
Autonomous Cortisol Secretion

By definition, an adrenal incidentaloma is an asymptomatic adrenal mass detected on imaging not performed for suspected adrenal disease. In most cases, adrenal incidentalomas are nonfunctioning adrenocortical adenomas, but may also represent conditions requiring therapeutic intervention (e.g. adrenocortical carcinoma, pheochromocytoma, hormone-producing adenoma or metastasis). The purpose of this guideline is to provide clinicians with best possible evidence-based recommendations for clinical management of patients with adrenal incidentalomas based on the GRADE (Grading of Recommendations Assessment, Development and Evaluation) system. We predefined four main clinical questions crucial for the management of adrenal incidentaloma patients, addressing these four with systematic literature searches: (A) How to assess risk of malignancy?; (B) How to define and manage low-level autonomous cortisol secretion, formerly called ‘subclinical’ Cushing’s syndrome?; (C) Who should have surgical treatment and how should it be performed?; (D) What follow-up is indicated if the adrenal incidentaloma is not surgically removed?Selected recommendations:(i) At the time of initial detection of an adrenal mass establishing whether the mass is benign or malignant is an important aim to avoid cumbersome and expensive follow-up imaging in those with benign disease. (ii) To exclude cortisol excess, a 1mg overnight dexamethasone suppression test should be performed (applying a cut-off value of serum cortisol ≤50nmol/L (1.8µg/dL)). (iii) For patients without clinical signs of overt Cushing’s syndrome but serum cortisol levels post 1mg dexamethasone >138nmol/L (>5µg/dL), we propose the term ‘autonomous cortisol secretion’. (iv) All patients with ‘(possible) autonomous cortisol’ secretion should be screened for hypertension and type 2 diabetes mellitus, to ensure these are appropriately treated. (v) Surgical treatment should be considered in an individualized approach in patients with ‘autonomous cortisol secretion’ who also have comorbidities that are potentially related to cortisol excess. (vi) In principle, the appropriateness of surgical intervention should be guided by the likelihood of malignancy, the presence and degree of hormone excess, age, general health and patient preference. (vii) Surgery is not usually indicated in patients with an asymptomatic, nonfunctioning unilateral adrenal mass and obvious benign features on imaging studies. We provide guidance on which surgical approach should be considered for adrenal masses with radiological findings suspicious of malignancy. Furthermore, we offer recommendations for the follow-up of patients with adrenal incidentaloma who do not undergo adrenal surgery, for those with bilateral incidentalomas, for patients with extra-adrenal malignancy and adrenal masses and for young and elderly patients with adrenal incidentalomas

Resistant hypertension after shockwave lithotripsy: the rude awakening of an adrenal incidentaloma

2020 ◽  
Vol 13 (8) ◽  
pp. e235261
Author(s):  
Ramon Jr Bagaporo Larrazabal ◽  
Harold Henrison Chang Chiu ◽  
Mark Anthony Santiago Sandoval
Keyword(s):  
Blood Pressure ◽  
Cushing’S Syndrome ◽  
Adrenal Mass ◽  
Adrenal Incidentaloma ◽  
Signs And Symptoms ◽  
Cushing's Syndrome ◽  
Flank Pain ◽  
Shockwave Lithotripsy ◽  
Left Flank ◽  
Left Flank Pain

A 41-year-old woman presented to the hospital because of left flank pain. CT scan of the kidneys revealed left-sided calculi and an incidental right adrenal mass, no other symptoms noted. She then underwent shockwave lithotripsy (SWL). However, immediately postoperatively, she had elevated blood pressure and remained hypertensive despite having four different medications. How SWL could have increased blood pressure could not be identified. On endocrine consult 16 months after SWL, she was found to now exhibit signs and symptoms of Cushing’s syndrome. Further workup revealed the adrenal incidentaloma to be cortisol-secreting. After undergoing right laparoscopic adrenalectomy, her blood pressure normalised, cortisol levels decreased and signs of Cushing’s syndrome gradually improved. We hypothesise that the performance of the SWL could have triggered the adenoma to ‘awaken’ from being non-functioning to cortisol-producing since this was the only intervening event. Observations of other patients are needed to validate our hypothesis.

scholarly journals Insulin sensitivity in patients with adrenal incidentaloma

2006 ◽  
Vol 134 (7-8) ◽  
pp. 315-319 ◽  
Author(s):  
Miomira Ivovic ◽  
Svetlana Vujovic ◽  
Zorana Penezic ◽  
Milos Zarkovic ◽  
Milka Drezgic
Keyword(s):  
Insulin Sensitivity ◽  
Adrenal Mass ◽  
Adrenal Incidentaloma ◽  
Regular Insulin ◽  
Tolerance Test ◽  
Adrenal Tumors ◽  
Control Group ◽  
Adrenal Incidentalomas ◽  
Imaging Methods ◽  
Frequent Use

Introduction. Frequent use of modern imaging methods (such as ultrasound, CT and MRI) results in high incidence of accidentally discovered adrenal mass. Adrenal incidentalomas are accidentally discovered adrenal tumors by imaging methods without any prior suspicion of adrenal disease. Some studies have shown decreased insulin sensitivity in patients with adrenal incidentaloma. Objective. The objective of our study was to assess the insulin sensitivity in patients with adrenal incidentalom a. Method. A total of 22 patients with accidentally discovered adrenal mass confirmed by CT/MRI were evaluated in our study. Average age was 53.31?26.5 years and average BMI 25.84?3.65 kg/m2. Control group consisted of 33 healthy subjects. Insulin sensitivity was assessed by short ITT (insulin tolerance test). Blood samples were taken before, 3, 6, 9, 12, 15, 20 and 30 minutes after i.v. bolus of regular insulin (0.05 IU/kg BW). Glycemia was determined by glucose oxidase method. Statistical analysis was done by ANCOVA, using BMI as covariate. Results. Our results showed significantly lower insulin sensitivity in patients with adrenal incidentalomas comparing to the control group (4.95?0.58 vs. 6.62?0.47, p=0.015). Conclusion. Our patients with adrenal incidentalomas manifested lower insulin sensitivity what suggested further follow up and assessment of insulin sensitivity during endocrine evaluation of these patients.

scholarly journals SAT-212 Management of VHL-Associated Pheochromocytoma in Pregnancy

2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Andrew Reed Reikes ◽  
Ruchi Desai
Keyword(s):  
Blood Pressure ◽  
Heart Rate ◽  
Adrenal Mass ◽  
Case Series ◽  
Normal Serum ◽  
Beta Blockade ◽  
Adrenergic Blockade ◽  
Intrauterine Pregnancy ◽  
Imaging Characteristics ◽  
In Pregnancy

Abstract Background: The appropriate treatment of pheochromocytoma during pregnancy is crucial because, while its incidence is rare (0.007%), it is associated with increased maternal and fetal mortality1. Clinical Case: A 32 year old Caucasian primigravida female presented with intrauterine pregnancy at 25 weeks for evaluation and management of a 9 mm left adrenal mass. The patient was previously diagnosed at age 10 with Von-Hippel Lindau (VHL) syndrome and genetic testing was positive for a VHL gene mutation (Type 2C VHL). At age 14, she underwent laparoscopic adrenalectomy to excise a right sided pheochromocytoma. At age 20, imaging revealed an enhancing left adrenal mass with imaging characteristics consistent with pheochromocytoma and with catecholaminergic labs that were normal or only mildy increased to 1.5 times upper normal range. Pre-partum the patient had been experiencing periodic episodes of headaches and palpitations. She reported experiencing baseline levels of palpitations and sweating with new onset of pre-syncope and hypotension during her pregnancy. Labs revealed normal serum levels of fractionated free metanephrines. Alpha adrenergic blockade, beta adrenergic blockade, and surgery were considered but deemed unnecessary as the patient was asymptomatic, imaging indicated the left adrenal mass was stable in size, and the patient wished to avoid lifelong adrenal replacement therapy. The patient’s blood pressure and heart rate were monitored closely throughout pregnancy and the use of alpha blockade or beta blockade was not needed. The patient had an uncomplicated induced delivery at 39 weeks. MRI with contrast of abdomen performed 10 months postpartum confirmed the presence of an enhancing 8 mm lesion on the inferior pole of the left adrenal gland with imaging characteristics again consistent with pheochromocytoma. Conclusion: This case demonstrates that a conservative approach of monitoring blood pressure and heart rate can be sufficient and appropriate for management of some patients with pheochromocytoma and VHL during pregnancy. Citation: 1. ENDOCRINOLOGY IN PREGNANCY: Pheochromocytoma in pregnancy: case series and review of literature in: European Journal of Endocrinology Volume 177 Issue 2 (2017).

Intraoperative Hemodynamic Instability and Diagnosis of Pheochromocytoma During Excision of Adrenal Incidentaloma With Incomplete Workup: A Case Report

2017 ◽  
Vol 22 (3) ◽  
pp. 328-331 ◽  
Author(s):  
Anthony Chang ◽  
George Silvay ◽  
Andrew Goldberg
Keyword(s):  
Blood Pressure ◽  
Heart Rate ◽  
Case Report ◽  
Asymptomatic Patient ◽  
Preoperative Evaluation ◽  
Adrenal Incidentaloma ◽  
Hemodynamic Instability ◽  
Missed Diagnosis

Preoperative evaluation of incidentalomas for pheochromocytoma is imperative. This case report describes a scheduled adrenalectomy in an asymptomatic patient with what was eventually determined to be an incomplete biochemical workup. The intraoperative course was complicated by labile and rapid increases in blood pressure and heart rate, suggesting the missed diagnosis of pheochromocytoma. It is important for anesthesiologists to ensure adequate preoperative biochemical workup before excluding the possibility of coexisting pheochromocytoma.

scholarly journals Fever of Unknown Origin: Could It Be a Pheochromocytoma? A Case Report and Review of the Literature

2018 ◽  
Vol 2018 ◽  
pp. 1-6
Author(s):  
Uzma Mohammad Siddiqui ◽  
Stephany Matta ◽  
Mireya A. Wessolossky ◽  
Richard Haas
Keyword(s):  
Case Report ◽  
Adrenal Medulla ◽  
Adrenal Mass ◽  
Tumor Necrosis ◽  
Fever Of Unknown Origin ◽  
Clinical Presentation ◽  
Unknown Origin ◽  
Adrenal Incidentalomas ◽  
Extensive Investigation ◽  
Review Of The Literature

Pheochromocytomas are rare tumors that arise from the adrenal medulla, with an incidence of less than 1 per 100,000 person-years. These tumors are characterized by excess catecholamine secretion and classically present with the triad of headaches, palpitations, and sweating episodes. However, the clinical presentation can be quite variable. Herein, we present a patient who presented with persistent fevers. An adrenal mass was incidentally discovered during the extensive investigation for the fever of unknown origin. Consequently, blood and urine tests were done and found to be consistent with a pheochromocytoma. The resection of this pheochromocytoma resulted in resolution of fevers. It is hypothesized that fevers in patients with pheochromocytomas occur due to the excess catecholamine or possibly due to interleukins. This clinical presentation serves as a learning point that adrenal incidentalomas in the setting of fever of unknown origin should not be ignored. It also reminds clinicians that pheochromocytomas which present with fevers may have tumor necrosis and many such patients are at risk for multisystem crises.

scholarly journals A Rare Adrenal Incidentaloma That Mimics Adrenocortical Carcinoma

2018 ◽  
Vol 2018 ◽  
pp. 1-4 ◽  
Author(s):  
Kedar Lavingia ◽  
Ramyar Torabi ◽  
Samuel W. Kim ◽  
Marybeth S. Hughes ◽  
Eric C. Feliberti ◽  
...  
Keyword(s):  
Adrenocortical Carcinoma ◽  
Clinical Presentation ◽  
Adrenal Incidentaloma ◽  
Ct Imaging ◽  
Adrenal Incidentalomas ◽  
Liver Transaminase ◽  
Biochemical Evaluation ◽  
Histopathologic Analysis ◽  
Work Up ◽  
Elevated Liver Transaminase

Objective. We present a case of an adrenal hemangioma, an uncommon cause of an adrenal mass, and review the clinical presentation, work-up, and management of adrenal incidentalomas. Background. A 64-year-old male was found to have a right adrenal incidentaloma during work-up for elevated liver transaminase levels, later found to be from hepatitis C. The mass was suspicious for adrenocortical carcinoma on CT imaging. Biochemical evaluation revealed no evidence of function. He underwent an open right adrenalectomy. The mass was found to be an adrenal hemangioma on histopathologic analysis. Methods. This is a case report with pertinent review of the diagnosis and management of adrenal incidentalomas. Results. Adrenal hemangiomas are rare, benign, nonfunctional tumors typically found during imaging for other reasons. As illustrated by this case, they appear similar to adrenocortical carcinoma on CT imaging. The diagnosis is usually not made prior to surgical resection. Conclusion. Adrenal hemangioma is a rare nonfunctional adrenal incidentaloma that displays atypical features on CT imaging. The suspicion for adrenocortical carcinoma usually prompts adrenalectomy.

scholarly journals Atypical Adrenocortical Incidentaloma Causing Autonomous Cortisol Secretion and Cardiovascular Complications

2021 ◽  
Vol 5 (Supplement_1) ◽  
pp. A118-A118
Author(s):  
Salem Gaballa ◽  
Areeka Memon ◽  
Marigny Roberts
Keyword(s):  
Diabetes Mellitus ◽  
Myocardial Infarction ◽  
Blood Pressure ◽  
Type 2 Diabetes ◽  
Adrenal Mass ◽  
Adrenal Incidentaloma ◽  
Cortisol Secretion ◽  
Autonomous Cortisol Secretion ◽  
Urine Cortisol

Abstract Introduction: Autonomous cortisol secretion (ACS) is found in approximately 10% of patients with an adrenocortical incidentaloma (AI). The majority of these cases are due to non-ACTH-dependent ACS. 2% of patients with AI have adrenocortical cancer. ACS is associated with cardiovascular morbidity, includes arterial stiffness, hypertension, coronary heart disease, and fatal or nonfatal myocardial infarction. Case presentation: A 62-year-old Caucasian male with a history of hypertension (HTN), hyperlipidemia (HLD) and poorly controlled type-2 diabetes mellitus (T2DM) and a recent myocardial infarction (MI) status post (s/p) coronary artery bypass graft (CABG) who presented to the endocrine clinic for evaluation of left adrenal mass. Family history was significant for adrenal carcinoma of his brother at the age of 60. The patient denied any facial flushing, palpitations, tremors, heat or cold intolerance, abnormal sweating, diarrhea, constipation, abdominal pain, recent weight change. Physical examination was unremarkable except midline sternal scar s/p CABG. Vital signs were remarkable for a blood pressure of 142/90. Labs were unremarkable. CT adrenal revealed a left adrenal mass 4.9 x 3.5 x 2.3 cm with HU -3 to 28 heterogeneous. HbA 1C was 8.2 %. Adrenal incidentaloma workup revealed a normal 24-hour urine cortisol level of 29 mcg, abnormal mid-night salivary cortisol of 0.13 mcg/dL and 0.31 mcg/dL, elevated cortisol after 1 mg dexamethasone suppression test (DST) of 343 mcg/dL, elevated cortisol after 8 mg DST of 90 mcg/dL, normal metanephrines of 10 pg/mL and normal normetanephrine of 49 pg/mL, normal aldosterone of 6.1 ng/dL, normal renin activity of 0.36 ng/ml/hr (Aldo/ PRA ratio 16). ACTH was 4.5 pg/ml. The endocrine surgeon was consulted for left adrenalectomy. The pathology showed atypical adrenal cortical neoplasm which does not clearly fulfill the criteria for adrenal cortical carcinoma confirmed by two pathologists. Upon six months follow-up, a 24-hour urine cortisol level was normal, 1mg overnight DST was normal, and no evidence of left adrenal on adrenal CT. Discussion: Adrenal incidentaloma (AI) is a common endocrine diagnosis affecting ~2% of the general population. Up to 10% of patients with AI have autonomous secretion of adrenal hormones. Pheochromocytoma and autonomous cortisol secretion should be excluded in every case and aldosteronism in patients with underlying hypertension and/or hypokalemia. ACS in patients AI has been associated with hypertension, insulin resistance, type 2 diabetes mellitus, obesity, metabolic syndrome, coronary artery disease, and increased mortality. Adrenalectomy is recommended for patients with a functional unilateral adenoma, with clinically significant hormone excess. Metabolic improvement after adrenalectomy, including weight loss, blood pressure lowering, glucose tolerance, lower lipids have been reported.

Approach to the patient with adrenal incidentaloma

2021 ◽  
Author(s):  
Irina Bancos ◽  
Alessandro Prete
Keyword(s):  
Adrenal Mass ◽  
Young Patients ◽  
Adrenal Incidentaloma ◽  
Current Evidence ◽  
Adrenal Tumors ◽  
Cortisol Secretion ◽  
Adrenal Incidentalomas ◽  
Cross Sectional ◽  
Imaging Characteristics ◽  
Autonomous Cortisol Secretion

Abstract Adrenal tumors are commonly discovered incidentally, on cross-sectional abdominal imaging performed for reasons other than adrenal mass. Incidence of adrenal tumors increased 10-fold in the last two decades, with most diagnosed in older adults. In any patient with a newly discovered adrenal mass determining whether the adrenal mass is malignant and whether it is hormonally active is equally important to guide the best management. Malignancy is diagnosed in 5-8% of patients with adrenal tumors, with a higher risk in young patients, if history of extra-adrenal malignancy, in those with large adrenal tumors with indeterminate imaging characteristics, and in bilateral adrenal tumors. While overt hormone excess is uncommon in adrenal incidentalomas, mild autonomous cortisol secretion can be diagnosed in up to 30-50% of patients. As autonomous cortisol secretion is associated with increased cardiovascular morbidity and metabolic abnormalities, all patients with adrenal incidentalomas require work up with dexamethasone suppression test. Management of adrenal tumors varies based on etiology, associated comorbidities, and patient’s preference. This article reviews the current evidence on the diagnosis and evaluation of patients with adrenal mass and focuses on management of the most common etiologies of adrenal incidentalomas.

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