Adrenal Neoplasia

2015 ◽  
Author(s):  
Anand Vaidya
Keyword(s):  
Adrenocortical Carcinoma ◽  
Adrenal Mass ◽  
Screening Tests ◽  
Management Approach ◽  
Adrenal Steroidogenesis ◽  
Counseling And Testing ◽  
Genetic Mechanisms ◽  
Adrenal Masses ◽  
Clinical Determination ◽  
Neuroendocrine Organ

The adrenal glands are composed of two distinct tissue types: the cortex, which serves as a factory for adrenal steroidogenesis, and the medulla, which produces catecholamines as a neuroendocrine organ. Neoplasia of the adrenal is approached by considering both whether the tumor is benign or malignant and whether it may represent a hormonally active tumor that can contribute to cardiometabolic disease or a hormonally silent tumor. Adrenal neoplasia is rarely malignant. This module discusses the approach to an incidentally discovered adrenal mass, with emphasis on the clinical determination as to whether it is benign or malignant and hormonally functional or nonfunctional. The pathogenesis and genetics of hyperaldosteronism and pheochromocytoma-paraganglioma are reviewed, as are the pathogenesis and management of adrenocortical carcinoma. Tables describe the differential diagnosis and diagnostic approach for an incidentally discovered adrenal mass; suggested biochemical screening tests for incidentally discovered adrenal masses; radiographic features of adrenal masses to determine benign or malignant potential; and the genetics of primary hyperaldosteronism, pheochromocytoma, and paraganglioma syndromes. A drawing shows the genetic mechanisms of hyperaldosteronism in familial hyperaldosteronism type III. Algorithms outline the suggested management approach for the incidentally discovered adrenal mass, genetic counseling and testing for patients with pheochromocytoma or paraganglioma, testing for family members of patients with pheochromocytoma and positive genetic testing, patients with stage I–III adrenocortical carcinoma, and patients with advanced adrenocortical carcinoma. This module contains 6 highly rendered figures, 6 tables, 55 references, and 5 MCQs.

Could we assess the functional status, of hormone secreting, or non-secreting of the adrenal masses regarding their Magnetic Resonance Imaging (MRI) characteristics?

2021 ◽  
Vol 22 ◽  
Author(s):  
Gamze Akkus ◽  
Ferhat Piskin ◽  
Barış Karagun ◽  
Murat Sert ◽  
Mehtap Evran ◽  
...  
Keyword(s):  
Magnetic Resonance Imaging ◽  
Magnetic Resonance ◽  
Chemical Shift ◽  
Functional Status ◽  
Signal Intensity ◽  
Adrenal Mass ◽  
Resonance Imaging ◽  
Intensity Index ◽  
Adrenal Masses ◽  
Magnetic Resonance Imaging Mri

Background: Diagnostic imaging techniques including magnetic resonance imaging (MRI) should also perform on all patients with incidentalomas. However, there is a limited study whether the quantitative measurements (signal intensity index, adrenal to spleen ratio) in MRI could predict the functional status of adrenal adenomas. Material-Method: Between 2015-2020; 404 patients (265 females, 139 males) with adrenal mass who were referred to the university hospital for further investigation were included. After detailed diagnostic hormonal evaluation, all patients underwent MRI 1.5 T device (Signa, GE Medical Systems; Milwaukee, USA). The signal intensities of the adrenal lesions on T2W images were qualitatively evaluated and noted as homogenous or heterogeneous in comparison with the liver signal intensity (SI). A chemical-shift SI index and chemical shift adrenal-to-spleen SI ratio were also calculated. Results: While 331(81.9%) of the patients had nonfunctional adrenal mass, the rest of them (n=73, 18.1%) were patients with functional (autonomous cortisol secretion-ACS, cushing syndrome-CS, pheochromocytoma, primary hyperaldosteronism-PA) adrenal masses. In phase vs phase values of patients with NFAI, Pheo(n=17), ACS (n=30), CS (n=11), and PA (n=15) were 474.04±126.7 vs 226.6±132.4, 495.3±182.8 vs 282.17±189.1, 445.2±134.8 vs 203.3±76.2, 506.8±126.5 vs 212.2±73.6 and 496.2±147.5 vs 246.6±102.1, respectively. Mean signal intensity index (SII) and adrenal to spleen ratio (ASR) of all groups (NFAI, Pheo, ACS, CS, PA) were 52.0±24.8 and 0.51, 44.9±22.5 and 0.55, 49.5±24.5 and 0.53, 56.2±16.4 and 0.43, 47.6±25.1 and 0.54, respectively. Based the current accepted measurements in the case of ASR and SII, all lesions were similar and shown as fat rich adenomas (p*= 0.552, p** = 0.45). Conclusion: The quantitative assessment (SII, ASR) of intracellular lipids in an incidentally discovered adrenal tumour could only help distinguish adrenal masses in case of adenomas or non-adenomas As initial diagnostic evaluation, clinical and laboratory assessment ,to distinguish hormone secretion, should be taken in all patients with adrenal incidentalomas.

scholarly journals A Case Report on Bilateral Adrenal Histoplasmosis

2020 ◽  
Vol 23 (2) ◽  
pp. 210-212
Author(s):  
Md Sazzad Hossain ◽  
Mohammad Zahid Hasan ◽  
Mir Ehteshamul Haque ◽  
Mostakim Maoya
Keyword(s):  
Adrenal Mass ◽  
Adrenal Glands ◽  
Histoplasma Capsulatum ◽  
Needle Aspiration ◽  
Immunosuppressed Patient ◽  
Aspiration Cytology ◽  
Fine Needle ◽  
Disseminated Histoplasmosis ◽  
Adrenal Masses ◽  
Needle Aspiration Cytology

Disseminated histoplasmosis affecting the adrenal glands is a very rare infection and an uncommon cause of adrenal insufficiency. We present a case of bilateral adrenal histoplasmosis in a 77 years old immunosuppressed patient who presented with bilateral adrenal masses. The fine needle aspiration cytology of the adrenal mass was positive for Histoplasma capsulatum. Bangladesh Journal of Urology, Vol. 23, No. 2, July 2020 p.210-212

scholarly journals SUN-180 A Case of Deoxycorticosterone-Producing Malignant Adrenocortical Tumor

2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Natasha Scaranello Cartolano ◽  
Vânia Balderrama Brondani ◽  
Amanda Meneses Ferreira Lacombe ◽  
Helaine Charchar ◽  
Bruna Pessoa ◽  
...  
Keyword(s):  
Weight Loss ◽  
Arterial Hypertension ◽  
Ct Scan ◽  
Adrenocortical Carcinoma ◽  
Adrenal Mass ◽  
Laboratory Data ◽  
Pulmonary Nodules ◽  
Adrenocortical Tumor ◽  
Abdominal Ct ◽  
Weiss Criteria

Abstract Background: Hypermineralocorticism (hypertension, hypokalemia, and low plasma renin activity) due to deoxycorticosterone (DOC) excess associated with adrenocortical carcinoma is extremely rare. DOC-producing tumors cause primary aldosteronism-like symptoms presenting low plasma aldosterone with very high DOC levels, and due to weak hormonal DOC activity, its diagnostic is done lately. Generally, malignant cases are progressive with a dismal prognosis. Clinical case: A 61-year-old woman was admitted to our hospital presenting lumbar pain and weight loss of 8 kg, in 2018. Previously, arterial hypertension was diagnosed in 2015, showing a satisfactory control with two classes of antihypertensive drugs. Physical exam: The patient presented no features of Cushing syndrome, but a palpable abdominal mass was noticed in the right flank. Blood pressure was 160x100 mmHg, with sustained high levels, despite regular treatment. Laboratory data: a hypokalemia (K 2.4 mEq/L, nr 3.5 -5.0 mEq/L) and hypernatremia (Na 146 mEq/L, nr 135 to 145 mEq/L), with metabolic alkalosis (venous pH 7.46 and serum bicarbonate 32 mmol/L, nr 23-27 mmol/L) was confirmed. Hormonal tests excluded hypercortisolism and pheocromocytoma. Serum aldosterone and renin were suppressed. Mineralocorticoid precursors dosage was extremely high, DOC (654 ng/dL, nr < 25 ng/dL) and progesterone (5.0 ng/mL, nr <0.89 ng/mL), as well 11-deoxycortisol (7.2 ng/mL, nr <0.5 ng/mL). Radiological imaging: abdominal CT showed a heterogeneous hypervascular adrenal mass (13.0x13.0x21.0 cm) exhibiting central necrosis, suggesting malignancy. FDG-PET/CT scan showed a hypermetabolic adrenal mass (SUVmax=13.8). Also, two metabolically active pulmonary nodules (SUVmax=3.7) measuring 0.7 and 0.4 cm were detected. The patient underwent right adrenalectomy, and the tumor was removed (24x13x13 cm). According to Weiss criteria (8/9) and modified Weiss criteria (5/7), the tumor was considered an adrenocortical carcinoma. Immunohistochemistry revealed a low Ki-67 index (10%). After the surgical procedure, all adrenal steroid levels normalized, and mitotane was prescribed as adjuvant therapy. Although the pulmonary nodules were stable at the four-month follow-up, the abdominal CT-scan revealed a heterogeneous nodule (3.7cm) in the left adrenal gland, which was suspicious of metastasis. Conclusion: DOC-producing adrenocortical tumors are heterogeneous regarding tumor size, clinical behavior, hormonal and metabolites secretion, and disease-free and overall survival; however, it is common hypokalemia, hypertension, and other symptoms as abdominal pain, due to tumor growth, and weight loss. The association of arterial hypertension with hypokalemia and elevated 11-deoxycortisol, with normal aldosterone and renin, lead to the need for mineralocorticoid precursors evaluation in patients with adrenocortical tumor.

Properly collected plasma metanephrines excludes PPGL after false positive screening tests

2021 ◽  
Author(s):  
Gregory A Kline ◽  
Jessica Boyd ◽  
Brenda Polzin ◽  
Adrian Harvey ◽  
Janice L Pasieka ◽  
...  
Keyword(s):  
False Positive ◽  
Adrenal Mass ◽  
Chart Review ◽  
False Positive Rate ◽  
Total Positivity ◽  
Screening Tests ◽  
Positive Rate ◽  
Plasma Metanephrines ◽  
Positive Results ◽  
Urine Metanephrines

Abstract Context False positive results are common for pheochromocytoma/paraganglioma(PPGL) real-world screening. Objective Determine the correlation between screening urine and seated plasma metanephrines in outpatients where PPGL was absent, compared to meticulously prepared and supine-collected plasma metanephrines with age-adjusted references. Design Retrospective cohort study Setting Databases from a single-provider provincial laboratory(2012-2018), a validated PPGL registry and a manual chart review from a specialized endocrine testing unit. Patients PPGL registry data excluded known PPGL cases from the laboratory database. Outpatients having both urine and plasma metanephrines <90 days apart. Methods The correlation between urine and seated plasma measures along with the total positivity rate. All cases of plasma metanephrines drawn in the endocrine unit were reviewed for test indication and test positivity rate. Results There were 810 non-PPGL pairs of urine and plasma metanephrines in the laboratory database; 46.1% of urine metanephrines were reported high. Of seated outpatient plasma metanephrines drawn a median of 5.9 days later, 19.2% were also high (r=0.33 and 0.50 for normetanephrine and metanephrine, respectively). In contrast, the meticulously prepared and supine collected patients(n=139, 51% prior high urine metanephrines) had <3% rate of abnormal high results in patients without known PPGL/adrenal mass. Conclusions There was a poor-to-moderate correlation between urine and seated plasma metanephrines. Up to 20% of those with high urine measures also had high seated plasma metanephrines in the absence of PPGL. Properly prepared and collected supine plasma metanephrines had a false positive rate of <3% in the absence of known PPGL/adrenal mass.

scholarly journals Open adrenalectomy for medium sized adrenocortical tumour: How I do it?

2015 ◽  
Vol 9 (5-6) ◽  
pp. 291 ◽  
Author(s):  
Wael M. Sameh ◽  
Ahmed Fouad Kotb
Keyword(s):  
Adrenocortical Carcinoma ◽  
Tumour Volume ◽  
Lateral Approach ◽  
Easy Access ◽  
Surgical Removal ◽  
Residual Tumour ◽  
Respiratory Problems ◽  
Adrenal Masses ◽  
The Mean ◽  
Adrenocortical Tumour

Introduction: The aim of our work was to report our experience in managing cases with medium-sized adrenocortical carcinoma by the high retroperitoneal extra pleural approach.Methods: During the past 2 years, 10 patients with suspected adrenocortical carcinoma were managed by our technique: the high supra 10th rib, retroperitoneal extra pleural approach. We included cases with 5 to 10 cm adrenal masses, suspected as adrenocortical carcinoma.Results: The mean patient age was 38 years (range: 26–44), the median tumour volume was 7 cm (range: 5–8). Of the 10 patients, 7 were female. Of the patients, 6 had right- and 4 had left-sided tumours. Intraoperatively, all cases had proper surgical removal, with no apparent residual tumour tissue. No single patient required a chest tube or developed respiratory problems. There were no major vascular injuries during surgery. We did not compare our findings to the standard lateral or subcostal approaches, as in our institution we adopt this high lateral approach for medium-sized tumours, while managing larger tumours with transperitoneal subcostal approach and smaller tumours laparoscopically.Conclusion: The high supra 10th lateral retroperitoneal, extra pleural approach is a safe, doable technique, allowing easy access to medium-sized suprarenal tumours and its vasculature, for cases suspected to be adrenocortical carcinoma.

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

scholarly journals Transcutaneous biopsy of adrenocortical carcinoma is rarely helpful in diagnosis, potentially harmful, but does not affect patient outcome

2014 ◽  
Vol 170 (6) ◽  
pp. 829-835 ◽  
Author(s):  
Andrew R Williams ◽  
Gary D Hammer ◽  
Tobias Else
Keyword(s):  
Patient Outcome ◽  
Overall Survival ◽  
Adrenocortical Carcinoma ◽  
Stage I ◽  
Adrenal Biopsy ◽  
Significant Difference ◽  
Adrenal Masses ◽  
Affect Patient Outcome ◽  
Rare Malignancy ◽  
The University

ContextAdrenocortical carcinoma (ACC) is a rare malignancy with high recurrence and mortality rates. The utility, sensitivity, and effect on patient outcome of transcutaneous adrenal biopsy (TAB) for single, large, adrenal masses are unclear.ObjectiveThis study evaluated the utility, diagnostic sensitivity, and effect on patient outcome of TAB in patients with ACC.Design and settingWe conducted a retrospective review of the electronic medical records of all ACC patients who were evaluated at the University of Michigan Health System from 1991 to 2011. We evaluated the sensitivity of TAB for tumors with the final pathological diagnosis of ACC. We compared the characteristics and survival of patients with stage I–III disease who underwent TAB with those who did not undergo TAB.ResultsA total of 75 ACC patients with TAB were identified. Complications occurred in at least 11% of patients and were mainly associated with bleeding. The maximum sensitivity of the procedure in diagnosing ACC was 70%. For stage I–III patients, baseline characteristics, stage at diagnosis, and adjuvant treatment with mitotane or radiation were not significantly different between the TAB (n=36) and the non-TAB (n=254) groups. There was no significant difference in recurrence-free (P=0.7) or overall survival (P=0.7) between patients who underwent TAB and those who did not.ConclusionsTAB of single, large, adrenal masses is usually unnecessary, exposes patients to risk, but does not affect recurrence-free or overall survival.

scholarly journals SAT-207 Adrenocortical Carcinoma in Untreated Congenital Adrenal Hyperplasia

2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Julia David ◽  
Lev Yagudayev ◽  
Robert Galagan
Keyword(s):  
Abdominal Pain ◽  
Congenital Adrenal Hyperplasia ◽  
Cortisol Level ◽  
Adrenocortical Carcinoma ◽  
Adrenal Mass ◽  
Dexamethasone Suppression Test ◽  
Adrenal Hyperplasia ◽  
Tumor Transformation ◽  
Suppression Test ◽  
Dexamethasone Suppression

Abstract Background: Congenital adrenal hyperplasia (CAH) is a group of rare inherited autosomal recessive disorders characterized by a deficiency of various enzymes participating in steroid hormone synthesis. It occurs in 1 in 5000 to 1 in 15000 births. The most common (90-95%) cause of CAH is the absence of the enzyme 21-hydroxylase. We are presenting a case of a 34 year old male with untreated congenital adrenal hyperplasia due 21-hydroxylase deficiency and metastatic adrenocortical carcinoma. Case: A 34 year old male with a history of classic congenital adrenal hyperplasia (CAH) untreated since childhood presented with symptoms of right abdominal pain, anorexia and weight loss. His family history was significant for one brother diagnosed with CAH, father with liver and pancreatic cancer and another brother with colon cancer. After 2 weeks of treatment with NSAIDs for the pain, his abdominal pain worsened and patient also reported dyspnea with minimal exertion and dry cough. The patient was admitted to TUMC and his evaluation revealed a large right adrenal mass (17x11cm) with multiple liver and lung lesions suggestive of metastatic cancer. The adrenal mass was needle biopsied and pathology results were diagnostic of adrenal cortical carcinoma. His baseline morning cortisol level was 22.45 mcg/dL, ACTH 13.2 (n=7.2-63.3), androstenedione 291 ng/dL (n=27-152), 17-hydroxy progesterone 10,850 ng/dL (n=27-199), testosterone 140 mg/dL, renin 1.295 ng/ml/h (n=0.167-5.38), aldosterone unable to assay due to interference and LDH 2011 U/L (n=87-241). He failed an overnight 1mg dexamethasone suppression test with cortisol of 20.17 in the morning. During hospitalization, his clinical condition gradually deteriorated with hypotension, altered mental status, acute respiratory failure and acute liver failure with an AST 2787 units/L (n <39), ALT 399 (n=30-65) and ALP 1013 units/L (n=40-120). Oncology decided that the patient was a poor candidate for antineoplastic treatment therefore he was offered hospice care and eventually expired. Discussion: There have been reports of benign and malignant adrenal tumors in patients with CAH. It has been surmised that ACTH is the driver of adrenal tumor transformation in these patients. Our patient with adrenocortical carcinoma presented at a late stage with widespread metastases resulting in death. His elevated cortisol level occurred in association with low normal ACTH and the failed 1mg overnight dexamethasone suppression test are consistent with tumor production of cortisone. Considering that he had untreated CAH since childhood, we assume elevated ACTH levels were present until tumor transformation occurred.

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