Surgical anesthesia in a patient with a pheochromocytoma crisis supported by ECMO: a case report.

Pheochromocytoma is rare in clinical practice, with patients typically presenting with headache, high blood pressure and sweating. Patients who develop a pheochromocytoma crisis are particularly rare. This report describes the case of a patient in a pheochromocytoma crisis who presented with severe cardiogenic shock, acute respiratory failure, and acute coronary syndrome.

Hereditary leiomyomatosis and renal cell cancer (HLRCC), pheochromocytoma (PCC)/paraganglioma (PGL) and germline fumarate hydratase (FH) variants.

Summary: Hereditary leiomyomatosis and renal cell cancer (HLRCC) is an autosomal dominant condition characterized by multiple cutaneous and uterine leiomyomas and renal cell cancer (RCC). HLRCC is caused by germline pathogenic/likely pathogenic (P/LP) variants in the fumarate hydratase (FH) gene on chromosome 1q42.3, encoding the mitochondrial enzyme responsible for the conversion of fumarate to malate in the Krebs cycle.

Unexpected pheochromocytoma leading to cardiac arrest during the perioperative period: a case report and literature review.

Background: Pheochromocytomas (PCCs) are rare neuroendocrine catecholamine (CA)-secreting tumours that originate from chromaffin tissue and can produce and store CAs. Unexpected PCCs pose a serious threat to the perioperative safety of patients and a considerable challenge to anaesthesiologists because of the risks of fatal hypertensive crises and other stresses.

Case Presentation: A 37-year-old woman who was scheduled for tonsillectomy and palatopharyngoplasty under general anaesthesia experienced a malignant cardiovascular event after induction, which was characterized mainly by a sharp increase in heart rate and blood pressure, ultimately leading to cardiac arrest and the occurrence of secondary long QT syndrome.

Long-Term Cardiovascular Complications in Patients With Pheochromocytomas and Paragangliomas After Surgery: A Large Multi-Center Study.

Objective: The effects of pheochromocytomas and paragangliomas (PPGLs)-induced catecholamine overproduction on vascular and cardiac function are generally thought to be reversible after PPGLs removal. However, a sizable proportion of patients who were free of the recurrent disease still faced high risks of cardiovascular problems after successful surgery. We aim to identify incidence and risk factors for long-term cardiovascular complications in PPGLs patients after surgery.

Prognosis and tumor microenvironment in pseudohypoxic pheochromocytoma/paraganglioma.

Pheochromocytoma and paraganglioma (PPGL) are rare tumors that occur in the adrenal medulla and extra-adrenal tissues, respectively. The prognosis and tumor microenvironment (TME) of pseudohypoxic PPGL as a major entity have not been fully described. Based on the clinical database of 65 patients with PPGL, we assessed the morphological features as well as the immunohistochemistry of pseudohypoxia-related proteins (SDHB and CAIX) and TME-related immune cell markers.

Successful analgesic treatment with continuous sacral epidural ethanol injection therapy for anal pain caused by multiple metastases of malignant pheochromocytoma.

Background: Anal and perineum pain caused by malignant tumor invasion is often difficult to control with opioids. Continuous sacral epidural ethanol injection therapy is less likely to cause bladder and rectal disturbances, making it a suitable treatment option for patients with preserved voiding function.

Case Presentation: A 45-year-old woman with multiple metastases of malignant pheochromocytoma suffered severe anal pain that worsened, especially when sitting, and was unresponsive to opioid rescue therapy.

Shedding light on biochemical changes in single neuron-like pheochromocytoma cells following exposure to synchrotron sourced terahertz radiation using synchrotron source Fourier transform infrared microspectroscopy.

Synchrotron sourced Fourier transform infrared (SS FTIR) microspectroscopy was employed to investigate the biological effects on the neuron-like pheochromocytoma (PC 12) cells after exposure to synchrotron sourced terahertz (SS THz) radiation. Over 10 min of exposure, the PC 12 cells received a total energy of 600 J m, with a total incident power density of ∼1.0 W m (0.

Insights into the genetic landscape of pheochromocytomas and paragangliomas in a Brazilian cohort.

Objective: Germline and somatic drivers are identified in 30% and 40% of pheochromocytomas and paragangliomas (PPGLs), respectively. In this study, we investigated the genetic landscape of PPGLs in a Brazilian cohort.

Methods: We studied 182 index patients with PPGLs (116 females and 66 males), comprising 118 pheochromocytoma and 70 paraganglioma cases.

Diaphragmatic pheochromocytoma: Two case reports and a review of the literature.

Rationale: Pheochromocytomas typically arise in the adrenal medulla, whereas ectopic pheochromocytomas/paragangliomas commonly occur near the abdominal aorta, bladder, mediastinum, and head. Diaphragmatic pheochromocytomas are exceedingly rare, and there is limited surgical experience with their treatment.

Patient Concerns: In Case A, the subject is a 45-year-old male, while in Case B, the subject is a 59-year-old female.

Hypotension with and Without Hypertensive Episodes During Endoscopic Adrenalectomy for Pheochromocytoma or Paraganglioma-Should Perioperative Treatment Be Individualized?

: Hemodynamic instability is common during adrenalectomy for pheochromocytoma and paraganglioma (PPGL). Most analyses focus on the risk factors for intraoperative hypertension, but hypotension is a frequent and undesirable phenomenon during PPGL surgery. This study aimed to analyze the risk factors for hypotensive episodes during the removal of PPGL, and whether these episodes are always associated with concomitant intraoperative hypertensive events.

Non-Susceptibility Gene Variants in Head and Neck Paragangliomas.

Head and neck paragangliomas (HNPGLs) are rare neoplasms that, along with pheochromocytomas and extra-adrenal paragangliomas, are associated with inherited mutations in at least 12 susceptibility genes in approximately 40% of cases. However, due to the rarity of HNPGLs, only a series of small-scale studies and individual cases have reported mutations in additional genes that may be involved in tumorigenesis. Consequently, numerous disease-causing mutations and genes responsible for the pathogenesis of HNPGLs remain poorly investigated.

Guilu Erxian Jiao remodels dendritic spine morphology through activation of the hippocampal TRPC6-CaMKIV-CREB signaling pathway and suppresses fear memory generalization in rats with post-traumatic stress disorder.

Ethnopharmacological Relevance: Guilu Erxian Jiao (GLEXJ) is a renowned traditional Chinese herbal formula used to tonify the kidney. It is employed to treat psychiatric disorders, and alleviate memory impairment, cognitive dysfunction, and behavioral disorders. Modern pharmacological studies have demonstrated GLEXJ's ability to significantly inhibit the fear response in post-traumatic stress disorder (PTSD) and facilitate the extinction of fear memory.

Is the TriNetX Database a Good Tool for Investigation of Real-World Management of Von Hippel-Lindau?

Von Hippel-Lindau (vHL) is a hereditary disease characterized by the development of benign and malignant tumors across multiple organ systems. It is seen in approximately 1 in 36,000 live births. Given that vHL is a rare disease, studies that seek to characterize vHL are often hampered by small sample sizes.

Benefits of transitioning from transperitoneal laparoscopic to retroperitoneoscopic adrenalectomy-a single center experience.

Background: Since 2017, switching from laparoscopic transabdominal adrenalectomy (LTA), posterior retroperitoneoscopic adrenalectomy (RPA) is used as standard procedure in this institution. Aim of this retrospective study was to compare both techniques regarding operative time, length of stay and safety of the procedures.

Methods: All patients operated in our institution for adrenal tumors were prospectively documented in the EUROCRINE-database and retrospectively analyzed.

An Incidental Finding of a Large Pheochromocytoma in an Asymptomatic Male: A Case Report.

Pheochromocytoma is a rare neuroendocrine tumor that secretes excess catecholamines. Patients present with a classical triad of headache, palpitations, and sweating. If untreated, pheochromocytoma can result in life-threatening cardiovascular complications.

Prevalence of the major thyroid cancer-associated syndromes in the United States.

Importance: A subset of thyroid cancers develops in a setting of a known hereditary cancerassociated syndrome. Understanding the population prevalence of thyroid cancer-associated syndromes is important to guide germline genetic testing and clinical management.

Objective: To estimate the prevalence of the major thyroid cancer-associated syndromes in the United States using the All of Us Research Program (AoU) data.

Atomoxetine-Induced Pheochromocytoma and Paraganglioma Crisis Managed With Veno-Arterial Extracorporeal Membrane Oxygenation.

Pheochromocytoma and paragangliomas (PPGLs) crises can be triggered by various factors, including norepinephrine reuptake inhibitors used to treat attention deficit hyperactivity disorder (ADHD), which worsen symptoms in patients with PPGLs. Therefore, attention should be paid to the potential for serious adverse reactions in patients with PPGLs taking ADHD medications. A 21-year-old man presented to the emergency department with acute onset of severe respiratory and circulatory failure after initiating atomoxetine treatment.

Pheochromocytoma-induced cardiogenic shock requiring ECMO: cardiovascular recovery prior to surgical resection.

An otherwise healthy woman in her 30s presented with cardiogenic shock and acute pulmonary oedema (APO), subsequently requiring extracorporeal membrane oxygenation (ECMO). A CT scan revealed an adrenal mass that prompted the differential diagnosis of a pheochromocytoma crisis that was later further suggested by raised plasma metanephrines. In the absence of clear guidelines due to the clinical rarity, a decision was made to delay adrenalectomy and stabilise the patient from a cardiac perspective with the view to operate after improvement of cardiac function with adequate alpha and beta blockade.

Phaeochromocytoma mimicking acute ST-elevation myocardial infarction.

Phaeochromocytomas are rare catecholamine-secreting tumours, usually benign, originating from chromaffin cells of the adrenal glands. Their typical presentation includes the triad of headaches, sweating and tachycardia due to excessive catecholamine release. However, many patients do not exhibit all three symptoms, making diagnosis challenging.

Genetic landscape of Romanian PPGLs.

Pheochromocytomas and paragangliomas (PPGLs) are rare neuroendocrine tumours that originate from chromaffin cells and occur in the adrenal medulla and in the sympathetic or parasympathetic ganglia. Nearly 70% of PPGLs result from germline or somatic mutations in a single driver gene. The aim of this study was to characterize the genetic background and clinical characteristics related to genetic profile of patients with PPGLs from Romania.

Perioperative outcomes of adrenal surgery: Does surgical specialty matter?

Introduction: Management of adrenal disease requires a multidisciplinary approach often involving varied specialists. Surgical management has often overlapped between general surgeons, usually with an interest in surgical endocrinology, or urologists with minimally invasive surgical skills. The objectives of this study were to define perioperative outcomes of contemporary Canadian adrenal surgery, and determine whether those outcomes are impacted by surgical subspecialty.

[F]AlF-NOTA-pentixather PET/CT of CXCR4 in patients with suspected primary hyperaldosteronism.

Distinguishing unilateral aldosterone-producing adenomas (APA) from idiopathic hyperaldosteronism (IHA), nonfunctional adrenal adenoma (NFA), and pheochromocytoma (PHEO) within primary aldosteronism (PA) presents a significant challenge. Studies have demonstrated high levels of chemokine receptor (CXCR) 4 expression in APA, thereby validating the use of Ga-labeled CXCR4 PET/CT for detecting APA. This study evaluates the efficacy of [F]AlF-NOTA-pentixather PET/CT in distinguishing APA from other PA types.

Mechanisms of resistance to RET-directed therapies.

The association between RET and multiple endocrine neoplasia type 2 was established in 1993 and remains one of the very few oncogenes for which distinct phenotypes (medullary thyroid cancer or phaeochromocytoma) are associated with the same hot-spot variants occurring either in germline or somatic DNA. Somatic RET fusion events have also been described in several cancers, including papillary thyroid cancer, non-small cell lung cancer, breast cancer, salivary gland cancer and pancreatic cancer. Highly selective RET inhibitors have improved outcomes in RET-altered cancers and have been well tolerated.

Partial biochemical response of adrenal artery embolization for pheochromocytoma: A case report and review of the literature.

We present the case of a 22-year-old male with a left adrenal pheochromocytoma, initially diagnosed during a workup for thoracic pain. The patient's tumor was refractory to medical management, and surgical resection was ruled out due to high cardiovascular risk, stemming from cyanotic congenital heart disease, aortic aneurysm, and factor VII deficiency. The patient underwent adrenal artery embolization (AAE) as a salvage treatment.