True mesothelial cyst of the adrenal gland: its clinical profile and management.

The incidence of adrenal cysts is 0.06% and only 9% of these are true mesothelial cysts. Here, we present a case of a true mesothelial cyst together with a review of the literature. A female in her 30s presented to the surgical outpatient department complaining of right flank pain. Her contrast-enhanced CT scan revealed a 7.5×6.5×4.5 cm right adrenal gland cyst. The patient underwent a laparoscopic right adrenalectomy. Immunohistopathology revealed the cyst to be mesothelial in nature. The majority of true mesothelial adrenal cysts are benign, unilateral and more common in women. Any adrenal cyst diagnosed as a functional lesion or one that may be malignant or with a diameter of 5 cm or greater requires surgical care whereas smaller lesions can be managed conservatively. Laparoscopic adrenalectomy for an adrenal cyst of diameter greater than 6 cm is a safe and feasible procedure in expert hands if there is no invasion of surrounding tissue.

Waterhouse-Friderichsen syndrome, septic adrenal apoplexy.

Waterhouse-Friderichsen syndrome is a rare but potentially fatal disorder of the adrenal gland characterized by bilateral adrenal hemorrhage. It is classically a result of meningococcal sepsis and presents acutely with features of shock, petechial rashes, abdominal pain, and non-specific symptoms such as headache, fatigue, and vomiting. Treatment consists of fluid resuscitation, corticosteroid replacement, and possibly surgery. The prognosis is poor despite treatment. This chapter will review the etiology, pathogenesis, clinical features, and management of the disease.

Surgical Technique and Perioperative Outcomes Following Single-Port Robotic Adrenalectomy: A Single Institutional Experience.

Objective: To describe a single institution's experience with single-port robotic adrenalectomy (SP-RA) and report perioperative outcomes. Materials and Methods: This is a retrospective, single-center study of adult patients who underwent SP-RA between January 2019 and April 2022 by a single surgeon using an IRB-approved institutional database. Patient demographics, perioperative data, surgical pathology, and postoperative outcomes were assessed. Results: Nineteen patients were identified who underwent SP-RA by a single surgeon over the period reviewed. One patient underwent bilateral SP-RA for bilateral adrenal masses, totaling 20 SP-RA procedures performed. Mean operative time was 80.7 ± 22.9 minutes and mean length of stay was 33.1 ± 27.9 hours. Two minor complications (Clavien-Dindo

Hypercortisolaemia without clinical stigmata of Cushing syndrome.

A man in his 20s was referred by his general practitioner because of the finding of adrenocorticotropic hormone (ACTH)-dependent hypercortisolaemia, discovered as part of investigation of fatigue and alopecia. The man had no other clinical findings suggestive of Cushing syndrome. Further investigation revealed intact diurnal rhythm in cortisol production, normal bone density and excluded assay interference. Further investigation revealed the man's sibling had been labelled as having Cushing syndrome because of similar biochemical abnormalities. A diagnosis of familial primary generalised glucocorticoid resistance syndrome was made. Testing for mutations in the NR3C1 gene is awaited.

Steroid profiling in adrenal disease.

The measurement of steroid hormones in blood and urine, which reflects steroid biosynthesis and metabolism, has been recognized as a valuable tool for identifying and distinguishing steroidogenic disorders. The application of mass spectrometry enables the reliable and simultaneous analysis of large panels of steroids, ushering in a new era for diagnosing adrenal diseases. However, the interpretation of complex hormone results necessitates the expertise and experience of skilled clinicians. In this scenario, machine learning techniques are gaining worldwide attention within healthcare fields. The clinical values of combining mass spectrometry-based steroid profiles analysis with machine learning models, also known as steroid metabolomics, have been investigated for identifying and discriminating adrenal disorders such as adrenocortical carcinomas, adrenocortical adenomas, and congenital adrenal hyperplasia. This promising approach is expected to lead to enhanced clinical decision-making in the field of adrenal diseases. This review will focus on the clinical performances of steroid profiling, which is measured using mass spectrometry and analyzed by machine learning techniques, in the realm of decision-making for adrenal diseases.

Bilateral Adrenal Infarction That Developed in Latent Essential Thrombocythemia.

Bilateral adrenal infarction is an extremely rare disease, and it has been reported that some coagulation abnormalities, including essential thrombocythemia (ET), exist in the background. We herein report a 76-year-old patient in whom the platelet count had been in the normal range at the onset of adrenal infarction but subsequently increased to 102×104/µL at 7 months later, leading to the diagnosis of JAK2V617F-positive ET. As the presence of the JAK2V617F mutation increases the risk of thrombosis, Janus kinase 2 (JAK2) genetic testing should be considered in some cases of nonspecific unknown thrombosis, even if there are no obvious hematological findings, such as clonal hematopoiesis of indeterminate potential (CHIP).

Bilateral adrenal hemorrhage: learning notes from clinical practice and literature review.

Adrenal hemorrhage is a rare, but important, diagnosis to recognize, in particular when there is involvement of both adrenal glands. Bilateral adrenal hemorrhage can in fact lead to adrenal insufficiency, with dramatic consequences if not promptly recognized and treated. It is normally caused by systemic conditions that lead to the vasoconstriction and thrombosis of the adrenal vein. Oftentimes, the clinical diagnosis of this condition can be very challenging, as its signs and symptoms are generalized and nonspecific (abdominal pain, nausea, and fatigue). Here, we present the cases of two patients admitted to the Emergency Department in 2016 and 2022 with acute abdominal pain, having recently undergone surgery and subsequently prescribed low-molecular-weight heparin. In both cases, laboratory results revealed neutrophilic leukocytosis and an unexplained anemia. Due to the persistence of abdominal pain despite medication, a CT scan was performed, showing an enlargement of both adrenal glands suggestive of bilateral adrenal hemorrhage. Adrenal function was tested that correlated with a diagnosis of adrenal insufficiency, and both patients were promptly treated with parenteral hydrocortisone as a result. On 5 years' follow-up from the acute event, the second patient's adrenal function had returned to normal, and he has not needed further adrenal replacement therapy; the first patient however demonstrated persistence of adrenal failure requiring replacement therapy. In this paper, through our experience and a literature analysis, we will aim to outline some clues to identify patients at potential risk of bilateral adrenal hemorrhage.

An unusual cause of abdominal pain: spontaneous bilateral adrenal hemorrhage.

Bilateral adrenal hemorrhage (BAH) is a rare condition that can lead to acute adrenal insufficiency and death if not recognized and treated promptly. We report the case of a 30-year-old male who presented to the emergency department with acute abdominal pain, nausea, and vomiting. On emergency room admission, the first abdominal CT revealed normal adrenal glands without enlargement, but with the development of hypotension and hypoglycemia, a second CT performed four days later showed enlargement due to hemorrhage in both adrenals. The diagnosis of BAH associated with acute adrenal insufficiency was retained. Prompt treatment with intravenous and oral corticosteroids resulted in successful conservative management. We describe the clinical, biological, radiological and etiological features of this condition based on a review of the literature.

Acute spontaneous non-hemorrhagic adrenal infarction in pregnancy: case-report and literature review.

Unilateral non-hemorrhagic adrenal infarction (NHAI) is a very uncommon cause of acute abdomen in pregnancy. Diagnosis is highly challenging due to its rarity, heterogeneity of clinical presentation, and inconclusiveness of the initial workup. Timely recognition is pivotal to ensuring optimal outcomes. Here we describe a case of spontaneous unilateral NHAI diagnosed in a singleton pregnant woman at 32 weeks' gestation at our centre and provide the findings of an extensive literature review on the topic. We identified 22 articles describing 31 NHAI cases in 30 obstetric patients: NHAI occurs more frequently on the right side and in the third trimester, and diagnosis is formulated more than 24 h after clinical presentation in 50% of cases; second-level imaging is always necessary to reach a definitive diagnosis and start appropriate treatment. A high degree of clinical suspicion is needed to promptly recognize NHAI in pregnancy, thus allowing appropriate multidisciplinary management and timely treatment initiation. Promotion of knowledge and awareness of NHAI as a potential cause of acute abdomen in pregnancy is mandatory to improve clinical practice and, ultimately, perinatal outcomes.

Giant adrenal gland pseudo-cyst: a case report with literature review.

Adrenal Gland Cysts are rare among all the pathologic cysts that occur in human beings; the pseudo-cyst variety even rarer. Adrenal pseudo-cysts are asymptomatic, non-functional, small, and incidentally discovered disease entities. Their clinical presentation is usually the result of their mass effects. Thanks to the advanced diagnostic technology, more such cases are being discovered timely and managed surgically, before life-threatening complications occur. Open surgical treatment remains the treatment of choice for giant cysts.

Primary adrenal insufficiency due to bilateral adrenal hemorrhage-adrenal infarction in a patient with systemic lupus erythematosus and antiphospholipid syndrome: case presentation and review of the literature.

Primary adrenal insufficiency (PAI) is a rare disease which represents the end stage of a destructive process involving the adrenal cortex. Occasionally it may be caused by bilateral adrenal hemorrhagic infarction in patients with antiphospholipid syndrome (APS). We herein report the challenging case of a 30-year-old female patient with systemic lupus erythematosus (SLE) and secondary APS who was admitted to the emergency department (ED) due to fever, lethargy, and syncopal episodes. Hyponatremia, hyperkalemia, hyperpigmentation, shock, altered mental status, and clinical response to glucocorticoid administration were features highly suggestive of an acute adrenal crisis. The patient's clinical status required admission to the intensive care unit (ICU), where steroid replacement, anticoagulation, and supportive therapy were provided, with a good outcome. Imaging demonstrated bilateral adrenal enlargement attributed to recent adrenal hemorrhage. This case highlights the fact that bilateral adrenal vein thrombosis and subsequent hemorrhage can be part of the thromboembolic complications seen in both primary and secondary APS and which, if misdiagnosed, may lead to a life-threatening adrenal crisis. High clinical suspicion is required for its prompt diagnosis and management. A literature search of past clinical cases with adrenal insufficiency (AI) in the setting of APS and SLE was conducted using major electronic databases. Our aim was to retrieve information about the pathophysiology, diagnosis, and management of similar conditions.

Closing the loop on adrenal health, dysfunction, and disease.

A wearable microdialysis device measures ultradian patterns of adrenal hormone secretion in humans at minute scale (Upton et al.).

Cystic lesions of the adrenal gland.

Cystic lesions of the adrenal glands are relatively uncommon and most of them are clinically silent. Though rarely associated with malignant changes, they may carry clinically detrimental consequences if misdiagnosed. Cystic adrenal lesions exhibit a broad histomorphological spectrum, ranging from pseudocysts, endothelial cysts, epithelial cysts and parasitic cysts. Here we present the case of a young woman with left-sided abdominal pain and contrast-enhanced CT showing a 10.4×7.7×7.8 cm fluid-filled left suprarenal lesion. The patient underwent exploratory laparotomy with cyst excision, and the histopathological examination of the specimen revealed a pseudocyst of the left adrenal gland. Despite being rare, usually benign and asymptomatic, the diagnosis and management of these cystic lesions of the adrenal glands are often unclear. Any functional lesion, potentially malignant lesion or lesion more than 5 cm deserves surgical management, whereas others can be managed conservatively.

Acute adrenal insufficiency secondary to bilateral adrenal haemorrhage.

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