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In this case report, we describe a diagnosis of papillary renal cell carcinoma in a 76-year-old male patient who was incidentally found to have a left adrenal mass during routine aneurysm surveillance. Computed tomography demonstrated a left adrenal mass and left renal structure which was concerning for renal cell carcinoma. He underwent left adrenalectomy and initial histopathology demonstrated papillary renal cell carcinoma. He subsequently underwent left radical nephrectomy with lymph node dissection. Histopathological analysis of the removed left renal and nodal specimens revealed papillary renal cell carcinoma with lymph node metastasis. However, re-review of the adrenal pathology slides determined the specimen as represented by primary kidney tumor and not adrenal metastasis. This report reviews the presentation and radiological findings of synchronous papillary renal cell carcinoma and differential diagnosis for indeterminate adrenal mass on computed tomography.

Background: Teratomas are germ cell tumors derived from totipotent cells, with presentation outside the gonads being rare, and adrenal location being even rarer. Prenatally detected adrenal teratomas are extremely uncommon. Case report: We report a rare case of a 5-month-old girl with a prenatally detected left suprarenal mass, which was initially suspected to be a congenital neuroblastoma. Abdominal computed tomography showed a cystic lesion measuring 7.9 x 8.8 × 11.5 cm in the left suprarenal region compatible with adrenal teratoma. The infant underwent laparotomy with excision of the lesion. The histopathological diagnosis was cystic immature teratoma. Conclusion: Although adrenal teratoma is extremely rare, it should be included in the clinical and radiologic differential diagnosis of prenatally detected suprarenal masses.

Purpose/Objective(s): Stereotactic body radiotherapy (SBRT) is an effective treatment for oligometastatic disease in multiple sites. However, the optimal radiation dose for long-term local control of adrenal metastases has yet to be determined. The aim of this study is to evaluate outcomes of adrenal SBRT and to evaluate factors that correlate with local control. Materials/Methods: After IRB approval, a retrospective data review of patients treated with SBRT for adrenal metastases at a medical center in Israel between 2015 and 2021 was conducted. A biological effective dose was calculated using an alpha beta ratio of 10. Kaplan Meier and Cox regression were calculated using SPSS software to describe the hazard ratio for local control and survival. Results: 83 cases of adrenal SBRT were identified. The average age was 67 (range 42–92 years old). Non-small cell lung cancer was the primary site in 44 % of patients. A total of 70 % of the patients had oligometastatic disease (less than five lesions), and the rest were polymetastatic, responding to systemic therapy with oligo progression in the adrenal. The average gross tumor volume (GTV) was 42 ml. Respiratory control was applied in 88 % of cases; 49.3 % used 4-D/ITV, and 38.5 % used breath-hold or continuous positive airway pressure (CPAP) with free breathing. On multivariable analysis, Dose above 75 Gy (biological effective Dose) (HR = 0.41, p = 0.031), Dose above 8 Gy per fraction (HR = 0.53p = 0.038), and breath-holds or CPAP (HR = 0.65, p = 0.047) were significant for local control. From multivariable analysis, we computed a predicted nomogram curve using seven clinical parameters to evaluate local control odds. Conclusion: In this single institution series reported to date, we found unilateral adrenal SBRT safe, yet bilateral treatment harbors a risk of adrenal insufficiency. Biological effective Dose > 75 Gy (BED), motion management with breath-hold or CPAP, and Dose per fraction > 8 Gy were the enhanced local controls. We propose a nomogram to help in decision-making regarding total Dose and Dose per fraction when treating adrenal SBRT.

Background: Contextual fear learning is heavily dependent on the hippocampus. Despite evidence that catecholamines contribute to contextual encoding and memory retrieval, the precise temporal dynamics of their release in the hippocampus during behavior is unknown. In addition, new animal models are required to probe the effects of altered catecholamine synthesis on release dynamics and contextual learning. Methods: We generated 2 new mouse models of altered locus coeruleus–norepinephrine (NE) synthesis and utilized them together with GRABNE and GRABDA sensors and in vivo fiber photometry to investigate NE and dopamine (DA) release dynamics in the dorsal hippocampal CA1 during contextual fear conditioning. Results: Aversive foot shock increased both NE and DA release in the dorsal CA1, while freezing behavior associated with recall of fear memory was accompanied by decreased release. Moreover, we found that freezing at the recent time point was sensitive to both partial and complete loss of locus coeruleus–NE synthesis throughout prenatal and postnatal development, similar to previous observations of mice with global loss of NE synthesis beginning postnatally. In contrast, freezing at the remote time point was compromised only by complete loss of locus coeruleus–NE synthesis beginning prenatally. Conclusions: Overall, these findings provide novel insights into the role of NE in contextual fear and the precise temporal dynamics of both NE and DA during freezing behavior and highlight complex relationships between genotype, sex, and NE signaling.

Adrenal myolipoma is a benign adrenal tumor which contains macroscopic amount of adipose tissue and usually hormonally inactive. However, functional adrenal myolipoma has also been reported in the literature.In this article, we present an interesting case of hormone secreting adrenal myelolipoma in an asymptomatic pregnant woman.

A 52-year-old male had pain in the right back and right hypochondrium, and an abdominal CT scan revealed a 49-mm tumor in the right upper perirenal space. Additional MRI and PET-CT suggested that the tumor may be a primary adrenal carcinoma and could invade the liver and diaphragmatic leg. The tumor was completely removed by laparotomy and histopathologically diagnosed as retroperitoneal primary undifferentiated pleomorphic sarcoma. The patient has remained recurrence-free for 1.5 years after the surgery.

There are a wide range of benign and malignant pathologies that the radiologist may encounter in the adrenal glands and kidneys, often incidentally when imaging is performed for other indications. Many imaging modalities including CT, MR, and US are often used in an attempt to characterize these lesions. A definitive radiological diagnosis, however, is not always possible. This is at times due to atypical presentations of typical lesions which may be mistaken for more aggressive or concerning pathologic conditions. Adrenal lesions that do not demonstrate characteristic benign imaging features might require surgical excision. Similarly, cystic renal lesions that demonstrate nodular enhancement are concerning for Bosniak IV lesions and require surgical management. We report 3 cases in 3 different patients of incidentally discovered hematomas with peripheral enhancement, 2 involving the adrenal gland and 1 involving the kidney. All 3 of these histologically proven hematomas demonstrated similar radiological manifestations of peripheral nodular progressive enhancement, mimicking neoplastic conditions, and necessitating surgical removal.

Primary adrenal leiomyosarcoma is a very rare mesenchymal tumor that arises from smooth muscle cells in the wall of the central adrenal vein or its branches (1). Less than 50 cases have been published in the English literature (2). The tumors are aggressive and often metastasize. This report describes a case of primary adrenal leiomyosarcoma that presented as intermittent left flank pain of 6 months duration in an otherwise healthy 58-year-old Caucasian female. The patient was initially imaged with an abdominal ultrasound, which revealed a left suprarenal mass. A follow-up CT of the abdomen and pelvis confirmed a malignant appearing left adrenal mass. A subsequent PET-CT demonstrated increased metabolic activity within the adrenal mass without evidence of metastasis. Biopsy proven metastasis eventually was detected on surveillance CT studies over the course of 2.5 years. Since this is such a rare malignancy, documenting its imaging findings with multiple modalities is of importance to add to the medical literature and help further characterize its imaging features.

Ectopic Cushing's syndrome (CS) is rare and difficult to cure when the source is elusive. Medical management is complex and often times contraindicated in the medically complex patient. We present a complicated case of ectopic CS where bilateral percutaneous microwave ablation (MWA) of the adrenal glands successfully cured hypercortisolism when surgery and medical therapies were contraindicated. A 71-year-old male was diagnosed with ectopic CS after adrenocorticotropic hormone-dependent hypercortisolism persisted after hypophysectomy despite a positive gradient of >3 on inferior petrosal sinus sampling. An ectopic source was not identified. Surgery and medical therapies were contraindicated due to comorbidities and drug interactions. Bilateral MWA of the adrenal glands was performed. Postprocedurally cortisol levels declined and the patient was clinically adrenally insufficient at 6 months. Bilateral MWA of the adrenal glands can prove to be an effective treatment option for ectopic CS when surgical resection and medical therapies are ineffective or contraindicated.

Background/Objective: Bilateral adrenal hemorrhage is a rare cause of adrenal insufficiency. Cases have been reported of acute adrenal crisis with bilateral adrenal hemorrhage during acute coronavirus disease of 2019 (COVID-19). Our objective was to report a delayed presentation of acute adrenal crisis with bilateral adrenal hemorrhage 2 months after COVID-19. Case Report: An 89-year-old man who was hospitalized for COVID-19 pneumonia 2 months prior presented with lethargy. He was disorientated and hypotensive to 70/50 mm Hg without improvement with intravenous fluids. According to his family, since his previous hospitalization for COVID-19, his mental status had continued to deteriorate, and he was no longer able to perform activities of daily living. A computed tomography scan of the abdomen revealed bilateral heterogeneous enlargement of the adrenal glands. Laboratory values were significant for an am cortisol level of 8.42 mcg/dL, a sodium level of 134 mEq/L, and a bicarbonate level of 17 mEq/L. He was treated intravenously with hydrocortisone 100 mg and showed rapid improvement. Discussion: It has been shown that COVID-19 disease may cause an increased risk of bleeding or thromboembolism. The exact frequency of bilateral adrenal hemorrhage secondary to COVID-19 is unknown. Although there are a handful of cases reported, there are none to our knowledge with a delayed presentation, as exhibited in our patient. Conclusion: The patient’s presentation was consistent with acute adrenal crisis due to bilateral adrenal hemorrhage from prior COVID-19 disease. We aimed to highlight the importance of clinicians being aware of adrenal hemorrhage and adrenal insufficiency as a possible delayed consequence in patients with a history of COVID-19.

Background/Objective: Nonclassic congenital adrenal hyperplasia (NCCAH) may be overlooked or mistaken for polycystic ovarian syndrome. Unlike congenital adrenal hyperplasia (CAH), the enzymatic activities of 21-hydroxylase or 11β-hydroxylase in NCCAH are not completely lost. In this case, NCCAH presented in a patient with CYP21A2 and CYP11B1 heterozygous mutations, one of which is a variant of unknown significance in CYP11B1. Case Report: A 30-year-old woman presented with a chief complaint of irregular menses and hirsutism. Previous medical history was significant for a prolactin level of 34.7 ng/mL (reference range, 2.0-23.0 ng/mL), a total serum testosterone level of 77 ng/dL (reference range, 25-125 ng/dL, not sex-specific), and a 2-mm × 3-mm pituitary lesion. An adrenocorticotrophic hormone stimulation test increased the 17-hydroxyprogesterone level from 444 ng/dL at baseline to 837 ng/dL at 60 minutes (baseline female reference range and stimulated reference ranges are 10-300 ng/dL and

Introduction: Pheochromocytomas are rare neuroendocrine tumors. Bilateral and metastatic pheochromocytoma in children is even rarer. We report a case of a 11-year-old girl presenting with malignant hypertension related to a bilateral and metastatic pheochromocytoma. Case presentation: We report the case of a 11-year-old male girl who presented with malignant hypertension. CT scan findings and urinary catecholamines dosage were consistent with bilateral pheochromocytoma. After stabilization of blood pressure, the patient underwent cortex sparing bilateral adrenalectomy and removal of two metastatic lymph nodes. Post-operatively, she showed complete resolution of hypertension and no steroids were required. Conclusion: Pheochromocytoma should be considered as a possible diagnosis in children presenting with malignant hypertension. Stabilize blood pressure is important part in the preoperative period. Adrenal sparing surgery showed promising outcomes in treating cases of bilateral disease.

Adolescence, the transition between childhood and adulthood, is characterized by rapid brain development in white matter (WM) that is attributed in part to rising levels in adrenal and gonadal hormones. The extent to which pubertal hormones and related neuroendocrine processes explain sex differences in WM during this period is unclear. In this systematic review, we sought to examine whether there are consistent associations between hormonal changes and morphological and microstructural properties of WM across species and whether these effects are sex-specific. We identified 90 (75 human, 15 non-human) studies that met inclusion criteria for our analyses. While studies in human adolescents show notable heterogeneity, results broadly demonstrate that increases in gonadal hormones across pubertal development are associated with macro- and microstructural changes in WM tracts that are consistent with the sex differences found in non-human animals, particularly in the corpus callosum. We discuss limitations of the current state of the science and recommend important future directions for investigators in the field to consider in order to advance our understanding of the neuroscience of puberty and to promote forward and backward translation across model organisms.

Cholesteryl ester (CE)-rich lipid droplets (LDs) accumulate in steroidogenic tissues under physiological conditions and constitute an important source of cholesterol as the precursor for the synthesis of all steroid hormones. The mechanisms specifically involved in CE-rich LD formation have not been directly studied and are assumed by most to occur in a fashion analogous to triacylglycerol-rich LDs. Seipin is an endoplasmic reticulum protein that forms oligomeric complexes at endoplasmic reticulum-LD contact sites, and seipin deficiency results in severe alterations in LD maturation and morphology as seen in Berardinelli-Seip congenital lipodystrophy type 2. While seipin is critical for triacylglycerol-rich LD formation, no studies have directly addressed whether seipin is important for CE-rich LD biogenesis. To address this issue, mice with deficient expression of seipin specifically in adrenal, testis, and ovary, steroidogenic tissues that accumulate CE-rich LDs under normal physiological conditions, were generated. We found that the steroidogenic-specific seipin-deficient mice displayed a marked reduction in LD and CE accumulation in the adrenals, demonstrating the pivotal role of seipin in CE-rich LD accumulation/formation. Moreover, the reduction in CE-rich LDs was associated with significant defects in adrenal and gonadal steroid hormone production that could not be completely reversed by addition of exogenous lipoprotein cholesterol. We conclude that seipin has a heretofore unappreciated role in intracellular cholesterol trafficking.

In this case report, we describe a diagnosis of papillary renal cell carcinoma in a 76-year-old male patient who was incidentally found to have a left adrenal mass during routine aneurysm surveillance. Computed tomography demonstrated a left adrenal mass and left renal structure which was concerning for renal cell carcinoma. He underwent left adrenalectomy and initial histopathology demonstrated papillary renal cell carcinoma. He subsequently underwent left radical nephrectomy with lymph node dissection. Histopathological analysis of the removed left renal and nodal specimens revealed papillary renal cell carcinoma with lymph node metastasis. However, re-review of the adrenal pathology slides determined the specimen as represented by primary kidney tumor and not adrenal metastasis. This report reviews the presentation and radiological findings of synchronous papillary renal cell carcinoma and differential diagnosis for indeterminate adrenal mass on computed tomography., (© 2024 The Authors. Published by Elsevier Inc. on behalf of University of Washington.)

Stereotactic ablative radiotherapy (SABR) planning for adrenal metastases aims to minimize doses to the adjacent kidney. Renal dose constraints for SABR delivery are not well defined. In 20 patients who underwent MR-guided breath-hold SABR in five daily fractions of 8–10 Gy, ipsilateral renal volumes receiving ≥20 Gy best correlated with loss of renal volumes, with median renal volume reduction being 6% (range: 3%-11%, 10th-90th percentiles). Organ function did not deteriorate in 18 patients, who had post treatment renal function tests available. This suggests that the ipsilateral renal volume receiving 20 Gy can be used as partial organ dose constraint for SABR to targets in the upper abdomen.

Retroperitoneal hematoma is a rare clinical entity with variable aetiology, which is increasing in incidence mainly due to complications related to interventional procedures. The causes of RE are different. We present 2 suggestive cases of RE, one for renal cause and another of adrenal origin. Both came to our attention as a matter of urgency. Retroperitoneal hematoma, therefore, originated from different causes, with consequent different treatments. Both cases were diagnosed, as a matter of urgency, thanks to the use of contrast Computed Tomography, which allowed a rapid diagnosis, careful specialist evaluation, a monitoring of their clinical conditions, and a consequent adequate outcome for the patients.

Summary: The classical model of the vital increase in systemic glucocorticoid availability in response to sepsis- and hyperinflammation-induced critical illness is one of an activated hypothalamus-pituitary-adrenocortical axis. However, research performed in the last decade has challenged this rather simple model and has unveiled a more complex, time-dependent set of responses. ACTH-driven cortisol production is only briefly increased, rapidly followed by orchestrated peripheral adaptations that maintain increased cortisol availability for target tissues without continued need for increased cortisol production and by changes at the target tissues that guide and titrate cortisol action matched to tissue-specific needs. One can speculate that these acute changes are adaptive and that treatment with stress-doses of hydrocortisone may negatively interfere with these adaptive changes. These insights also suggest that prolonged critically ill patients, treated in the ICU for several weeks, may develop central adrenal insufficiency, although it remains unclear how to best diagnose and treat this condition.

الملخص: سرطان الغدد الليمفاوية بيركيت، هو سرطان يصيب الغدد الليمفاوية اللاهودجكينية من النوع ب (الخلايا اللِّمفاوية أو اللِّمفاويَّات البائيَّة). انسداد الوريد الأجوف العلوي يُعتبر من النادر حدوثه مع سرطان الغدد الليمفاوية بيركيت، وهو غالبا يحدث مع الأنواع الأخرى من سرطان الغدد الليمفاوية اللاهودجكيِنية مثل سرطان الخلايا الكبيرة. نبلغ في هذا التقرير عن حالة نادرة لسرطان الغدد الليمفاوية بيركيت من النوع المتفرق مع انسداد الوريد الأجوف العلوي لدى صبي يبلغ من العمر ١٦ عاما مصابا بكتل سرطانية في البلعوم، المنصف الصدري والغدة الكظرية. المريض أتى بانتفاخ في الجزء الأيسر العلوي من الرقبة لمدة شهرين، مع ازدياد الحجم مع مرور الوقت بدون أيَة أعراض أخرى، وفي اليوم الثاني من التنويم تم اختبار المريض لفايروس كورونا (كوفيد-١٩) وكانت النتيجة إيجابية. في الفحص السريري كان لديه انتفاخ في الغدة اللمفاوية الرقبية وانتفاخان في الجهتين من الغدد اللمفاوية الأذنية الخلفية. لم يكن هناك شيئا ملحوظا في الاختبار الصدري باستثناء بعض الدوالي الوريدية على صدر المريض. لم يكن هناك أية أعراض أو علامات لانسداد الوريد الأجوف العلوي بغض النظر عن نتائج الأشعة المقطعية. بدأ المريض على العلاج الكيميائي المكثف، وكانت لديه استجابة ملحوظة للعلاج في الكتل السرطانية في البلعوم والمنصف الصدري، مع استجابة طفيفة للكتلة السرطانية في الغدة الكظرية. لم يكن هناك أيَة مضاعفات ملحوظة. Abstract: Burkitt's lymphoma (BL) is an aggressive non-Hodgkin B-cell lymphoma. Superior vena cava obstruction (SVCO) is considered a rare presentation of BL and it is usually associated with other types of non-Hodgkin lymphoma such as diffuse large-cell. We report a rare case of sporadic BL with SVCO in a 16-year-old boy with nasopharyngeal, mediastinal, and adrenal masses. The patient presented with a two-month history of left upper neck swelling that increased with time and was not associated with other symptoms. The patient tested positive for COVID-19 on the second day after admission. On examination, he had enlarged solitary lateral cervical and bilateral posterior auricular lymph nodes. There were no signs or symptoms of SVCO regardless of the findings suggested by the computed tomography of the chest. The patient was treated with hyper-CVAD chemotherapy and showed a remarkable resolution of the nasopharyngeal and mediastinal masses with a mild response of his adrenal mass. There were no complications detected during this patient's management.

ABSTRACT: Corticosterone is critical for the maturation and survival of chicken fetus around hatching. Betaine is used as a feed additive in poultry industry to promote growth and mitigate stress. However, it remains unknown whether betaine could affect adrenal corticosterone synthesis in pre-hatching chicken fetuses. In this study, betaine (2.5 mg/egg) was injected into developing chicken fetuses at d 11 of incubation (E11) and its impact on adrenal steroidogenesis was investigated at day 19 (E19). Plasma corticosterone concentration was significantly (P < 0.05) elevated in betaine-treated fetuses, together with increased adrenal expression of melanocortin 2 receptor and steroidogenic acute regulatory protein. Accordingly, the corticosterone biosynthetic enzymes, such as cytochrome P450 family 11 subfamily A member 1, 3β-hydroxysteroid dehydrogenase and cytochrome P450 family 21 subfamily A member 2, as well as cholesterol biosynthesis or regulation-related genes, such as sterol regulatory element-binding protein 1, 3-hydroxy-3-methyl-glutaryl-coenzyme A reductase and low-density lipoprotein receptor, were all significantly (P < 0.05) upregulated in betaine group. Meanwhile, steroidogenic factor-1 and glucocorticoid receptor were significantly (P < 0.05) enhanced, whereas expression of dosage-sensitive sex reversal-adrenal hypoplasia congenita critical region on the X chromosome gene, a nuclear receptor known as a repressor of adrenal steroidogenesis, was significantly (P < 0.05) downregulated. Betaine significantly (P < 0.05) increased adrenal expression of genes involved in one-carbon metabolism and DNA methylation, such as S-adenosyl homocysteine hydrolase, betaine-homocysteine-methyltransferase, methionine adenosyl transferase and DNA methyltransferases, yet the promoter regions of most steroidogenic genes were significantly (P < 0.05) hypomethylated. These results indicate that in ovo injection of betaine promotes adrenal glucocorticoid synthesis in chicken fetuses before hatching, which involves alterations in DNA methylation.

Objective: Dexamethasone is a known treatment for lymphoma, but the potency and rapidity of its effect have not been recognized. We present a case of bilateral adrenal lymphoma that significantly reduced in size after a single dose of dexamethasone. Methods: We present the clinical course and investigations, including adrenocorticotropic hormone, cortisol, short synacthen test, computed tomography (CT), and adrenal biopsy results. Results: A 52-year-old man had a fall and was incidentally found to have bilateral adrenal masses (left, 6 cm; right, 5 cm) on CT. His adrenal function tests included plasma metanephrines (normetanephrine, 830 pmol/L [normal,

Objective: In an adult endocrine clinic, the majority of patients referred for evaluation of an incidentally discovered adrenal mass are aged more than 30 years, for which many national and international societies have developed management guidelines. However, adrenal incidentalomas in children and young adults are uncommon. We report the case of an 18-year-old woman with an incidentally discovered right-sided adrenal mass. Methods: We present the adrenal tests, computed tomography, and magnetic resonance imaging results and treatment of a young woman with an adrenal mass that proved to be a ganglioneuroma. Results: A computed tomography scan showed a 2.2 × 2.6 cm right-sided adrenal mass with noncontrast Hounsfield units >10 and

Human cytochromes P45011β (CYP11B1) and P450aldo (CYP11B2) are monooxygenases that synthesize cortisol through steroid 11β-hydroxylation and aldosterone through a three-step process comprising 11β-hydroxylation and two 18-hydroxylations, respectively. CYP11B1 also catalyzes 18-monohydroxylation and 11β,18-dihydroxylation. To study the molecular basis of such catalytic divergence of the two enzymes, we examined a CYP11B1 mutant (Mt-CYP11B1) with amino acid replacements on the distal surface by determining the catalytic activities and crystal structure in the metyrapone-bound form at 1.4-Å resolution. Mt-CY11B1 retained both 11β-hydroxylase and 18-hydroxylase activities of the wild type (Wt-CYP11B1) but lacked 11β,18-dihydroxylase activity. Comparisons of the crystal structure of Mt-CYP11B1 to those of Wt-CYP11B1 and CYP11B2 that were already reported show that the mutation reduced the innermost space putatively surrounding the C3 side of substrate 11-deoxycorticosterone (DOC) bound to Wt-CYP11B1, while the corresponding space in CYP11B2 is enlarged markedly and accessible to bulk water through a channel. Molecular dynamics simulations of their DOC-bound forms supported the above findings and revealed that the enlarged space of CYP11B2 had a hydrogen bonding network involving water molecules that position DOC. Thus, upon positioning 11β-hydroxysteroid for 18-hydroxylation in their substrate-binding sites, steric hindrance could occur more strongly in Mt-CYP11B1 than in Wt-CYP11B1 but less in CYP11B2. Our investigation employing Mt-CYP11B1 sheds light on the divergence in structure and function between CYP11B1 and CYP11B2 and suggests that CYP11B1 with spatially-restricted substrate-binding site serves as 11β-hydroxylase, while CYP11B2 with spatially-extended substrate-binding site successively processes additional 18-hydroxylations to produce aldosterone.

Lynch Syndrome (LS), or hereditary non-polyposis colorectal cancer, is the most common cause of hereditary colorectal cancer. There are well described extra-colonic manifestations of LS, including gynecologic and upper urinary tract malignancies. Other extra-colonic manifestations of LS are less understood. Here we present an unusual case of a functional adrenal pheochromocytoma in a 31-year old man with LS.

We present a case of a middle-age male who presented in emergency room with nonspecific abdominal pain. A contrast-enhanced computer tomography (ceCT) scan showed a reduced perfusion of both adrenal glands. The clinical examinations and the laboratory tests were negative for an adrenal pathological process. To reassess the adrenal ischemia, a second ceCT scan was performed 5 days later showing an acute bilateral adrenal hemorrhage. These findings demonstrated that the previous adrenal hypoperfusion represented the prodromal manifestation of a hemorrhagic intraglandular process. This case suggests that adrenal hypoperfusion detected on tomographic imaging dictates a prompt clinical management finalized to strictly monitor the potential evolution towards a more aggressive pathological condition and confirms the pivotal role of imaging in the diagnosis of such uncommon disorder.

Adrenal gland myelolipomas are benign, hormonally inactive, and mostly asymptomatic and unilateral tumors. However, it could be symptomatic and bilateral in rare cases. The diagnosis is based on a CT scan and a histological study. We present a rare case of a surgically managed bilateral adrenal gland myelolipoma with a giant mass on the left side in a 40-year-old man who presented in our department for atypical abdominal pain. The patient underwent surgical resection of the left adrenal mass. Due to the resolution of the abdominal pain, a close follow-up for the right mass by CT scan was chosen.

Objectives: The evaluation of an adrenal mass is challenging. We present the case of a 33-year-old pregnant woman who was found to have an adrenal incidentaloma. Four months after the initial imaging, the mass vanished. Methods: We described the case of a pregnant woman with hypertension and an incidentally found right adrenal mass. Results: A magnetic resonance imaging scan showed a right adrenal mass measuring 7.9 × 3.9 × 3.0 cm with a multilobulated appearance. Initial biochemical testing was concerning for a pheochromocytoma with positive metanephrines during hospitalization while being treated for an infection. Repeat outpatient adrenal hormone results, including metanephrines, were negative. Four months after her initial magnetic resonance imaging scan, the right adrenal mass was no longer present. Conclusion: A 33-year-old pregnant woman was found to have a right adrenal mass that later vanished as a result of the resolution of a unilateral adrenal hemorrhage. Predisposing factors to adrenal hemorrhage in the presented case include pregnancy, infection, and hypertension.

Summary: Agonists for glucagon-like-peptide-1 receptor (GLP-1R) are currently used for the treatment of type 2 diabetes and obesity. Their benefits have been centered on pancreas and hypothalamus, but their roles in other organ systems are not well understood. We studied the action of GLP-1R on secretions of adrenal medulla. Exendin-4, a synthetic analog of GLP-1, increases the synthesis and the release of catecholamines (CAs) by increasing cyclic AMP (cAMP) production, without apparent participation of cAMP-regulated guanine nucleotide exchange factor (Epac). Exendin-4, when incubated for 24 h, increases CA synthesis by promoting the activation of tyrosine hydroxylase. Short incubation (20 min) increases the quantum size of exocytotic events by switching exocytosis from partial to full fusion. Our results give a strong support to the role of GLP-1 in the fine control of exocytosis.

Post-traumatic adrenal hematoma, which has rare but severe complications, is a difficult to diagnose condition. Only computed tomography, which is often performed systematically during lesional assessments of violent thoraco-abdominal trauma, can confirm the diagnosis. Symptoms are often masked by associated visceral or parietal lesions. Standard biological examinations are generally not very helpful. We will describe the case of a 47-year-old man who presented with a right adrenal hemorrhage following a road accident, for only associated lesion, a fracture of the middle arch of the first and 8th ribs, and the transverse process L1.

Offspring adrenal function may be negatively affected in utero by maternal stressors such as microbial infection. Maternal supplementation with immunomodulatory compounds such as omega-3 polyunsaturated fatty acids (n-3 PUFA) may help minimize the adverse effects of maternal stress on fetal hypothalamic-pituitary-adrenal development and improve offspring health. Presently, n-3 PUFA sources are primarily fish-based, but n-3 PUFA microalgae (AL) may be an alternative. Previously, it was determined that maternal AL or fish oil (FO) supplementation to sows, in addition to maternal stress induced by Escherichia coli lipopolysaccharide (LPS) challenge appeared to have a greater influence on the stress response of male offspring compared to females. To further elaborate on these findings, this study assessed the effects of maternal AL or FO supplementation combined with a maternal LPS challenge on adrenal gene expression in male offspring fed a nursery diet containing low-quality protein sources. Forty-eight sows were fed gestation diets starting on gestation day (gd) 75 containing either 3.12% AL, 3.1% FO, or a control diet containing 1.89% corn oil. On gd 112, half the sows in each treatment were administered 10 ​μg/kg LPS i.m. Piglets were weaned at 21 days of age onto a common low-quality plant-based protein diet, and one week after weaning, four piglets per sow were administered 40 ​μg/kg LPS i.m. Two hours later, the piglets were euthanized to obtain adrenal tissue, and total RNA was extracted to carry out transcriptome analysis using the Affymetrix GeneChip WT Plus assay and subsequent validation by real-time PCR. Analysis revealed that adrenal steroidogenesis, fatty acid metabolism and immune function were significantly influenced by maternal diet and stress. Increased expression of immune-related genes including lymphocyte antigen 96, TLR-2 and NF-κB suggests that maternal AL supplementation may increase offspring sensitivity to inflammation after weaning. Decreased expression of lymphocyte antigen 96 in male offspring from sows receiving maternal LPS challenge also suggests a possible role of maternal stress in diminishing the offspring immune response to immune stress challenge. Increased expression of the genes encoding the 11BHSD2 enzyme in offspring from sows fed FO may also reduce the magnitude of the stress response. These data provide insight to the immune and metabolic mechanisms that may be influenced by maternal diet and stress.

Microabstract: The effect of smoking on adrenal cancer is poorly understood. A clear association of adrenal adenoma and adrenocortical carcinoma with smoking among the United States population is observed. This association points to the possibility of environmental carcinogenic and/or lifestyle factors contributing to adrenal cancer formation. Our results support the association of tobacco use with adrenal adenomas and adrenal cortical carcinoma. Background: Smoking has been suggested as a risk factor for adrenal cortical carcinoma (ACC), but this hypothesis has only been inferred from a single study using all types of adrenal cancers including pheochromocytoma, neuroblastoma, as well as ACC. Given the high rate of tobacco use in West Virginia, we hypothesized that smoking might contribute to increased prevalence of ACC. Materials and Methods: De-identified institutional review board-exempted records were analyzed in the Surveillance, Epidemiology, and End Results (SEER) Program from 2001-2016 and in patients from the United States nationwide, multicenter TriNetX database of 41,063,707 patients from 2008-2018. In addition, the state-level ratio of smoking to ACC prevalence was computed in all 50 states using data from SEER and the Center for Disease Control. West Virginia Health System data from 2008-2018 was extracted to confirm population-level findings. Melanoma was used as a cancer control in both databases. Results: 6,946 ACC cases were identified. West Virginia had the highest smoking rate and the second highest rate of ACC. A significant association was found between smoking and ACC (Pearson correlation coefficient r = 0.4887, p=.0004). From 2008 to 2018 using TriNetX, 846 ACC and 36,434 AA were extracted. Both adrenal neoplasm cohorts had increased prevalence of tobacco use compared with melanoma controls, where 23.5% were smokers compared to 36.4% and 33.9% in the ACC and AA groups, respectively (p

Cholesterol is required for maintenance of plasma membrane fluidity and integrity and for many cellular functions. Cellular cholesterol can be obtained from lipoproteins in a selective pathway of HDL-cholesteryl ester (CE) uptake without parallel apolipoprotein uptake. Scavenger receptor B type 1 (SR-B1) is a cell surface HDL receptor that mediates HDL-CE uptake. It is most abundantly expressed in liver, where it provides cholesterol for bile acid synthesis, and in steroidogenic tissues, where it delivers cholesterol needed for storage or steroidogenesis in rodents. SR-B1 transcription is regulated by trophic hormones in the adrenal gland, ovary, and testis; in the liver and elsewhere, SR-B1 is subject to posttranscriptional and posttranslational regulation. SR-B1 operates in several metabolic processes and contributes to pathogenesis of atherosclerosis, inflammation, hepatitis C virus infection, and other conditions. Here, we summarize characteristics of the selective uptake pathway and involvement of microvillar channels as facilitators of selective HDL-CE uptake. We also present the potential mechanisms of SR-B1-mediated selective cholesterol transport; the transcriptional, posttranscriptional, and posttranslational regulation of SR-B1; and the impact of gene variants on expression and function of human SR-B1. A better understanding of this unique pathway and SR-B1's role may yield improved therapies for a wide variety of conditions.

Background: There are 4 prior reports of oncocytic pheochromocytoma of the adrenal. It may histologically resemble cortical carcinoma, which also can have oncocytic cells. Case presentation: We report a 54-year-old male with neurofibromatosis 1 who had an endocrinologically functional, 13.5 cm tumor. Histology showed mostly oncocytic cells. An adrenalectomy showed that the tumor had capsular and vascular invasion. Symptoms were cured by surgery. A cortical carcinoma with oncocytic change was initially favored, but the clinical history together with immunostain results were consistent with pheochromocytoma with oncocytic change. Conclusions: In conclusion, the clinical history was crucial to the diagnosis. This is the first tumor of this type in a neurofibromatosis patient and the first in which functional effects were caused.

Background: Preferred size-threshold recommendations for management of incidental adrenal lesions remain controversial., Purpose: This meta-analysis aimed to compare the diagnostic accuracy of different size thresholds for detecting malignancy in patients with incidental adrenal lesions on imaging., Materials and Methods: A systematic review of MEDLINE, Embase, Scopus, the Cochrane Library, and the gray literature, covering the period from inception to September 2021, was performed. Studies with >10 patients evaluating the diagnostic accuracy of imaging size thresholds for detecting malignancy in patients with incidental adrenal lesions and no prior history of cancer were included. Study, clinical, imaging, and accuracy data for eligible studies were independently acquired by two reviewers. Primary meta-analysis was performed using a bivariate mixed-effects regression model. Risk of bias was evaluated using the Quality Assessment of Diagnostic Accuracy Studies-2 (QUADAS-2) tool., Results: From 2,690 citations, 40 studies (9,794 patients with mean age ranging from 41 to 66 years) were included. Most (36 of 40) were retrospective single-center studies. CT with or without MRI served as the index test(s). Sensitivity and specificity values, respectively, by size threshold used in the included studies were as follows: 85% (95% confidence interval [CI] 74%-91%) and 39% (95% CI 23%-57%) for 3-cm thresholds; 85% (95% CI 78%-90%) and 75% (95% CI 62%-85%) for 4-cm thresholds; 70% (95% CI 56%-81%) and 74% (95% CI 59%-85%) for 5-cm thresholds; and 75% (95% CI 67%-82%) and 77% (95% CI 62%-87%) for 6-cm thresholds. No cause for variability in sensitivity or specificity was identified on subgroup analysis of the 4-cm threshold. Nearly half of the studies (19 of 40) had at least one QUADAS-2 domain with a high risk of bias., Conclusions: A 4-cm size threshold demonstrates the highest combined sensitivity and specificity, with a preserved specificity compared with higher size thresholds, but with a trend toward improved sensitivity. Future research reevaluating 4-5 cm size thresholds while excluding characteristically benign lesions by imaging may help redefine a size threshold that has improved specificity but preserved sensitivity, compared with the existing 4-cm threshold., (Copyright © 2023 American College of Radiology. Published by Elsevier Inc. All rights reserved.)

Primary aldosteronism (PA) is the most common form of secondary hypertension. Mutations in KCNJ5, ATP1A1, ATP2B3 and CACNA1D are found in aldosterone producing adenoma (APA) and familial hyperaldosteronism (FH). A recurrent mutation in CACNA1H (coding for Cav3.2) was identified in a familial form of early onset PA. Here we performed whole exome sequencing (WES) in patients with different types of PA to identify new susceptibility genes. Four different heterozygous germline CACNA1H variants were identified. A de novo Cav3.2 p.Met1549Ile variant was found in early onset PA and multiplex developmental disorder. Cav3.2 p.Ser196Leu and p.Pro2083Leu were found in two patients with FH, and p.Val1951Glu was identified in one patient with APA. Electrophysiological analysis of mutant Cav3.2 channels revealed significant changes in the Ca2+ current properties for all mutants, suggesting a gain of function phenotype. Transfections of mutant Cav3.2 in H295R-S2 cells led to increased aldosterone production and/or expression of genes coding for steroidogenic enzymes after K+ stimulation. Identification of CACNA1H mutations associated with early onset PA, FH, and APA suggests that CACNA1H might be a susceptibility gene predisposing to PA with different phenotypic presentations, opening new perspectives for genetic diagnosis and management of patients with PA.

There is strong evidence that the gonads modulate the hypothalamic–pituitary–adrenal axis. To investigate these sex differences at the adrenal glands of sheep we compared the cortisol response to ACTH (experiment 1) and measured the relative expression of oestrogen receptor alpha (ERS1), androgen receptor (AR), melanocortin 2 receptor (MC2R) and steroid acute regulatory protein (STAR) mRNA in adrenal glands (experiment 2) of gonadectomised rams and ewes either with or without sex steroid replacement. In experiment 1 six castrated adult rams and four ovariectomised adult ewes were used in two ACTH trials. On each trial blood samples were taken every 15 min for 4 h through an indwelling jugular catheter and each animal received 0.5 mg of an ACTH analogue i.v., immediately after the sample at 1 h from the beginning of the trial. Four days after the first trial the males received 100 mg of Testosterone Cyclopentilpropionate (TC) i.m. and the females received 2.5 mg of Oestradiol Benzoate (EB) i.m. At 72 h after TC or EB administration the second trial was performed. In experiment 2 the adrenal glands were obtained from gonadectomised adult rams (n=8) and adult ewes (n=8). Four rams received 100 mg of TC i.m. and four females received 0.5 mg of EB i.m. Blood samples were taken at 0, 12, 24, 48 and 72 h relative to steroid replacement and the animals were thereafter slaughtered. Cortisol, testosterone and 17β-oestradiol were determined by radioimmunoanalysis. The transcripts of ERS1, AR, MC2R and STAR were determined by real-time reverse transcription PCR in adrenal tissue. Cortisol secretion was higher in female sheep than in male sheep, and higher in EB-treated than non-treated ewes. No difference in cortisol secretion was observed between TC-treated and non-treated rams. Gonadectomised rams treated with TC presented greater AR mRNA and MC2R mRNA expression than males without the steroid replacement. Gonadectomised ewes treated with EB tended to present lower AR mRNA than the ones without steroid replacement. Gonadectomised rams with TC also had greater AR mRNA, ERS1 mRNA and MC2R mRNA expression than ewes treated with EB. The relative amount of STAR transcript was not different among the different groups. The results confirm sex differences in ACTH-induced cortisol secretion in sheep, as well as in the expression of the receptor proteins for both 17β-oestradiol and testosterone in the sheep adrenal gland. However, the underlying mechanisms for sex steroid modulation remain unresolved.

A retrospective review of the literature was performed to determine the natural history, prevalence, prognosis and management of adrenal injury associated with blunt abdominal trauma in pediatric population. Blunt adrenal injury in children is uncommon, rarely isolated, and typically present as part of a multi organ trauma. Adrenal hemorrhage is being diagnosed more frequently since the emergence of computed tomography in modern emergency rooms. Obstetric birth trauma during vaginal delivery of a macrosomic fetus may result in neonatal adrenal hemorrhage. In children appear to be an incidental finding that resolves on follow-up imaging. Most of these injuries are self-limited and do not require intervention. The differential diagnosis of an adrenal neoplasm, especially in children with an isolated adrenal hemorrhage, must be considered. The presence of adrenal hemorrhage in the absence of a trauma history should alert to the possibility of pediatric inflicted injury.

The mechanisms of HDL-mediated cholesterol transport from peripheral tissues to the liver are incompletely defined. Here the function of scavenger receptor cluster of differentiation 36 (CD36) for HDL uptake by the liver was investigated. CD36 knockout (KO) mice, which were the model, have a 37% increase (P = 0.008) of plasma HDL cholesterol compared with wild-type (WT) littermates. To explore the mechanism of this increase, HDL metabolism was investigated with HDL radiolabeled in the apolipoprotein (125I) and cholesteryl ester (CE, [3H]) moiety. Liver uptake of [3H] and 125I from HDL decreased in CD36 KO mice and the difference, i. e. hepatic selective CE uptake ([3H]125I), declined (–33%, P = 0.0003) in CD36 KO compared with WT mice. Hepatic HDL holo-particle uptake (125I) decreased (–29%, P = 0.0038) in CD36 KO mice. In vitro, uptake of 125I-/[3H]HDL by primary liver cells from WT or CD36 KO mice revealed a diminished HDL uptake in CD36-deficient hepatocytes. Adenovirus-mediated expression of CD36 in cells induced an increase in selective CE uptake from HDL and a stimulation of holo-particle internalization. In conclusion, CD36 plays a role in HDL uptake in mice and by cultured cells. A physiologic function of CD36 in HDL metabolism in vivo is suggested.

Pheochromocytomas are rare neuroendocrine tumors. Although predominantly occurring in the adrenal glands, these tumors can present anywhere along the sympathetic chain. Indeed, classical teaching states that 10% of pheochromocytomas are extra-adrenal and 10% are malignant. We report a case of a 61-year-old female who underwent presumptive cytoreductive nephrectomy and adrenalectomy for renal carcinoma but was instead found to have malignant pheochromocytoma. Proper identification, surgical extirpation, and follow-up are imperative for treatment. We review the classic and current literature regarding management of this uncommon tumor.

Se revisan los trastornos más comunes del sistema endocrino que se detectan en la consulta no especializada. Se hace énfasis en: a) Trastornos tiroideos tales como hipo o hipertiroidismo, nódulos de la tiroides y la importancia de la enfermedad de la tiroides durante el embarazo, b) la enfermedad adrenal en la hipertensión y el enfoque de la incidentaloma suprarrenal c) hiperparatiroidismo primario y la deficiencia de vitamina D d) Trastornos gonadal y la importancia de la detección precoz de la enfermedad hormonal, tanto en la disfunción ovárica y testicular.

Adolescence, the transition between childhood and adulthood, is characterized by rapid brain development in white matter (WM) that is attributed in part to rising levels in adrenal and gonadal hormones. The extent to which pubertal hormones and related neuroendocrine processes explain sex differences in WM during this period is unclear. In this systematic review, we sought to examine whether there are consistent associations between hormonal changes and morphological and microstructural properties of WM across species and whether these effects are sex-specific. We identified 90 (75 human, 15 non-human) studies that met inclusion criteria for our analyses. While studies in human adolescents show notable heterogeneity, results broadly demonstrate that increases in gonadal hormones across pubertal development are associated with macro- and microstructural changes in WM tracts that are consistent with the sex differences found in non-human animals, particularly in the corpus callosum. We discuss limitations of the current state of the science and recommend important future directions for investigators in the field to consider in order to advance our understanding of the neuroscience of puberty and to promote forward and backward translation across model organisms., Competing Interests: Declaration of Competing Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2023 The Authors. Published by Elsevier Ltd.. All rights reserved.)

Hypertension (HT) is a major cardiovascular risk factor that affects 10% to 40% of the general population in an age-dependent manner. Detection of secondary forms of HT is particularly important because it allows the targeted management of the underlying disease. Among hypertensive patients, the prevalence of endocrine HT reaches up to 10%. Adrenal diseases are the most frequent cause of endocrine HT and are associated with excess production of mineralocorticoids (mainly primary aldosteronism), glucocorticoids (Cushing syndrome), and catecholamines (pheochromocytoma). In addition, a few rare diseases directly affecting the action of mineralocorticoids and glucocorticoids in the kidney also lead to endocrine HT. Over the past years, genomic and genetic studies have allowed improving our knowledge on the molecular mechanisms of endocrine HT. Those discoveries have opened new opportunities to transfer knowledge to clinical practice for better diagnosis and specific treatment of affected subjects. In this review, we describe the physiology of adrenal hormone biosynthesis and action, the clinical and biochemical characteristics of different forms of endocrine HT, and their underlying genetic defects. We discuss the impact of these discoveries on diagnosis and management of patients, as well as new perspectives related to the use of new biomarkers for improved patient care., (Copyright © 2023 International Society of Nephrology. Published by Elsevier Inc. All rights reserved.)