Cardiovascular Degeneration in Erdheim-Chester Disease: Prevalence, Burden, and Outcomes.

Erdheim-Chester Disease (ECD) is a rare systemic histiocytic neoplasm, with cardiac morbidities, including cardiovascular (CV) risk factors and cardiac conditions, playing a significant yet poorly understood role in disease burden. This study evaluated the prevalence, characteristics, and prognosis of ECD in patients with (ECD-C) and without (ECD-noC) cardiac involvement and compared the burden of cardiac morbidities with matched controls. Patients diagnosed with ECD between 1990 and 2021 at a tertiary center were included, with cardiac involvement centrally assessed using radiographic studies.

Pituitary Photon Counting Detector CT for Cushing Disease: Pre-operative Lesion Localization, Intraoperative Findings, and Post-operative Outcomes.

Background And Purpose: MRI fails to localize a tumor in up to 40% of cases of ACTH dependent Cushing Disease (CD), impacting patient outcomes and creating uncertainty in the diagnosis. Photon-counting detector CT (PCD-CT) is a new CT technology with better imaging performance than conventional CT. PCD-CT is a novel imaging technique for CD, especially for MR-negative cases, however its application to CD remains unknown.

Approach to the Patient: From Endocrinopathy to the Diagnosis of a Histiocytic Disorder.

Endocrinopathies are frequently the initial presentation of histiocytic neoplasms, which are rare hematologic disorders affecting multiple organ systems. Langerhans cell histiocytosis and Erdheim-Chester disease are 2 such disorders known to infiltrate the hypothalamus and/or pituitary gland, leading to arginine vasopressin deficiency (AVP-D) and anterior pituitary dysfunction (APD) in 20% to 30% of cases, often as the first manifestation. Conversely, histiocytic disorders account for a notable proportion (10-15%) of all pituitary stalk lesions.

Performance of Dehydroepiandrosterone Sulfate and Baseline Cortisol in Assessing Adrenal Insufficiency.

Context: Diagnosing adrenal insufficiency (AI) often requires complex testing, which can be time-consuming and expensive. Dehydroepiandrosterone sulfate (DHEAS) is a promising marker of hypothalamic-pituitary-adrenal axis function; however, its diagnostic performance has not yet been evaluated in a large-scale study.

Objective: Evaluate the performance of DHEAS and baseline cortisol in assessing AI.

Soft tissue aneurysmal bone cyst presenting as an enlarging neck mass: Case report and review of the head and neck literature.

Soft tissue aneurysmal bone cysts (STABCs) are rare neoplasms histopathologically identical to aneurysmal bone cysts. These benign lesions are characterized by thin, peripheral ossification and no skeletal continuity. STABC may be difficult to distinguish from myositis ossificans (MO) and malignant entities from imaging and fine needle aspiration, due to rarity and overlapping features.

The clinical presentation of PIT1 positive pituitary neuroendocrine tumor immunonegative for growth hormone, prolactin, and thyroid stimulating hormone with analysis of clinical and immunostaining dissociation.

Background: PIT1 is a pituitary transcription factor that is associated with either growth hormone (GH), prolactin (PRL), or thyroid-stimulating hormone (TSH) production. However, PIT1-positive pituitary neuroendocrine tumors (PitNETs) are occasionally immunonegative for GH, PRL, and TSH. This paper describes the clinical presentation of PIT1 positive however immunonegative PitNETs.

Incidence of venous thromboembolism following adrenalectomy: A CESQIP analysis.

Background: We aim to evaluate the incidence of venous thromboembolism (VTE) following adrenalectomy.

Methods: A retrospective analysis of the Collaborative Endocrine Surgery Quality Improvement Program was performed to assess incidence for VTE, including pulmonary embolism or deep vein thrombosis, in adults undergoing adrenalectomy (2014-2022).

Results: 2567 patients undergoing adrenalectomy were included.

Long-term outcomes among adults with Langerhans cell histiocytosis.

Advances in the treatment of Langerhans cell histiocytosis (LCH) have resulted in a growing survivor population. There is a lack of data on long-term outcomes among adults with LCH. We conducted a retrospective record review of 219 adults (aged ≥18 years) with LCH.

Succinate dehydrogenase variants in paraganglioma: why are B subunit variants 'bad'?

Mutations that predispose to familial pheochromocytoma and paraganglioma include inherited variants in the four genes (, , and ) encoding subunits of succinate dehydrogenase (SDH), an enzyme of the mitochondrial tricarboxylic acid cycle and complex II of the electron transport chain. In heterozygous variant carriers, somatic loss of heterozygosity is thought to result in tumorigenic accumulation of succinate and reactive oxygen species. Inexplicably, variants affecting the SDHB subunit predict worse clinical outcomes.

Role of Multimodality Imaging and Preoperative Management in Intrapericardial Paragangliomas: Experience From a Case Series.

Intrapericardial paragangliomas are rare, highly vascular tumors that frequently adhere to adjacent structures and blood vessels, making surgical resection challenging. In this case series, we discuss the role of multimodality imaging and preoperative embolization in the management of 3 patients presenting with intrapericardial paragangliomas. ().

Secondary Adrenal Insufficiency: Recent Updates and New Directions for Diagnosis and Management.

Secondary adrenal insufficiency is the most common subtype of adrenal insufficiency; it is caused by certain medications and pituitary destruction (pituitary masses, inflammation, or infiltration) and is rarely associated with certain germline variants. In this review, we discuss the etiology, epidemiology, and clinical presentation of secondary adrenal insufficiency and focus on the diagnostic and management challenges. We also review the management of selected special populations of patients and discuss patient-important outcomes associated with secondary adrenal insufficiency.

Secondary Adrenal Insufficiency and Growth Hormone Deficiency in Patients with Fibromyalgia.

Purpose: Patients with fibromyalgia (FM) may demonstrate low cortisol concentrations during diagnostic evaluation. However, it remains unclear whether low cortisol reflects underlying pituitary dysfunction. We aimed to determine if a subset of patients with FM have concomitant secondary adrenal insufficiency (SAI) and growth hormone deficiency (GH).

Pheochromocytoma and Paraganglioma in Pregnancy: a New Era.

Purpose Of Review: Pheochromocytoma and paraganglioma (PPGL) in pregnancy is a rare entity and management of these patients is fraught with uncertainty. Our objective is to review current literature and discuss diagnosis and management of these patients.

Recent Findings: Outcomes of PPGL in pregnancy have improved in recent years.

The Role for Metyrosine in the Treatment of Patients With Pheochromocytoma and Paraganglioma.

Context: Treatment of pheochromocytoma and paraganglioma (PPGL) requires preintervention titration of alpha- and beta-adrenergic blockade, but patients may still be at risk for complications from catecholamine excess. Metyrosine decreases catecholamine production, making it an attractive therapeutic adjunct for select patients.

Evidence Acquisition: A systematic literature review was performed (Ovid Medline and Scopus databases) on December 17, 2019, including studies with humans and original data.

Not all adrenal incidentalomas require biochemical testing to exclude pheochromocytoma: Mayo clinic experience and a meta-analysis.

Background: Excluding a pheochromocytoma is important when a patient presents with an incidentally discovered adrenal mass. However, biochemical testing for pheochromocytoma can be cumbersome, time consuming, or falsely positive. Our objective was to determine if unenhanced computed tomography (CT) imaging alone can be used to rule out pheochromocytoma.

Bilateral pheochromocytoma: Clinical characteristics, treatment and longitudinal follow-up.

Objective: Comprehensive data about patients with bilateral pheochromocytoma are limited. We aimed to describe the clinical presentation, genetic analysis, treatment and outcomes of patients with bilateral pheochromocytoma.

Design: A retrospective study at a tertiary care centre.

Pheochromocytoma Characteristics and Behavior Differ Depending on Method of Discovery.

Context: Modern pheochromocytomas (PHEOs) are often discovered by incidental finding on cross-sectional imaging or mutation-based genetic case detection testing. Little is known about how these PHEOs behave.

Objective: To describe the characteristics and behavior of PHEOs discovered incidentally on imaging or through mutation-based genetic case detection testing.

Rotavirus Vaccine Schedules and Vaccine Response Among Infants in Low- and Middle-Income Countries: A Systematic Review.

Background: Rotavirus vaccine schedules may impact vaccine response among children in low- and middle-income countries (LMICs). Our objective was to review the literature evaluating the effects of monovalent (RV1) or pentavalent rotavirus vaccines schedules on vaccine response.

Methods: We searched PubMed, Web of Science, Embase, and ClinicalTrials.

Preoperative Levels of Catecholamines and Metanephrines and Intraoperative Hemodynamics of Patients Undergoing Pheochromocytoma and Paraganglioma Resection.

Objective: To determine whether, despite pharmacologic adrenergic receptor blockade, higher preoperative levels of catecholamines and metanephrines (adrenergic activity) are associated with increased intraoperative complications.

Materials And Methods: Records of patients undergoing paraganglioma and pheochromocytoma (PGL-PCC) resection from January 1, 2000, to June 30, 2015, were reviewed for preoperative levels of adrenergic activity, intraoperative variability in blood pressure and heart rate (range), and postoperative outcomes (hypotension requiring treatment). Patients were categorized by maximum preoperative adrenergic activity by greater degree of abnormality, categorized as normal (≤100%) or 101%-200%, 201%-500%, 501%-1000%, and ≥1001% of upper limit of normal.

Pheochromocytoma and paraganglioma in patients with neurofibromatosis type 1.

Objective: Individuals with neurofibromatosis type 1 (NF1) are at an increased risk of developing a pheochromocytoma or paraganglioma (PHEO/PGL). However, the best case detection strategy is unknown. Our objectives were to describe the prevalence, clinical presentation and outcomes of PHEO/PGL associated with NF1 and formulate case detection testing recommendations for PHEO/PGL.