Pheochromocytoma is the underlying etiology in 0.1% of hypertensive cases. However, it may be present in up to 5.7% of patients with neurofibromatosis I (NF1). The burst of catecholamines inherent in pheochromocytoma has significant effects on the mechanical and electrical activity of the myocardium. Different theories have been postulated for myocardial stunning in patients with pheochromocytoma that include microvascular spasm, impaired fatty acid metabolism, increased production of oxygen-derived free radicals and dynamic left ventricular mid-cavity obstruction. QT interval prolongation is seen in 16% to 35% of patients with pheochromocytoma. Takotsubo cardiomyopathy (TS) is now being increasingly identified and it may be responsible for up to 40% of cases of acute catecholamine cardiomyopathy. These manifestations may sometimes precede or cloud the typical triad of a headache, sweating, and tachycardia. We herein present a case of a 42-year-old female with a unique combination of QT prolongation, torsades de pointes, and TS caused by pheochromocytoma in the background of NF1. All these complications are potentially reversible with the removal of the underlying adrenal tumor, underscoring the importance of a high suspicion for pheochromocytoma in patients with NF1.
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http://dx.doi.org/10.7759/cureus.3646 | DOI Listing |
J Clin Endocrinol Metab
January 2025
Department of Endocrinology, Diabetology and Clinical Nutrition, University Hospital Zurich (USZ), and University of Zurich (UZH), CH-8091 Zurich, Switzerland.
Context: Pheochromocytomas and paragangliomas (PPGLs) are rare endocrine tumors that frequently produce catecholamines. Catecholamine-induced cardiometabolic complications substantially contribute to increased morbidity and mortality in PPGL patients prior to surgical resection.
Objective: To determine whether markers of elevated cardiometabolic risk persist in patients following PPGL resection.
Front Endocrinol (Lausanne)
January 2025
Department of Internal Medicine and Endocrinology, University Clinical Centre of the Medical University of Warsaw, Warsaw, Poland.
Clin Endocrinol (Oxf)
December 2024
Division of Endocrinology and Metabolism, Dokuz Eylul University Hospital, Izmir, Turkey.
Context: Neutrophil-to-lymphocyte ratio (NLR) and platelet-to-lymphocyte ratio (PLR) have recently emerged in assessing pheochromocytomas and paragangliomas (PPGLs). However, their combined use with PASS scale has not yet been explored.
Objective: Our goal was to investigate the prognostic values of NLR and PLR and incorporate the PASS score into our analysis.
Discov Oncol
December 2024
Department of Endocrinology, The First Affiliated Hospital of Chongqing Medical University, Chongqing, 400016, China.
Introduction: Although giant cystic pheochromocytoma and paraganglioma (PPGL) are uncommon, they can be life-threatening when it occurs. Unfortunately, prior case reports have shown that giant cystic PPGLs are highly susceptible to diagnostic errors. Therefore, this study aimed to explore giant cystic PPGLs by comparing them with non-cystic PPGLs, defining the clinical features of the affected patients, and analyzing the characteristics of misdiagnosis and mistreatment associated with PPGLs.
View Article and Find Full Text PDFFront Endocrinol (Lausanne)
December 2024
One Health Research Group, Universidad de las Americas, Quito, Ecuador.
Pheochromocytomas and paragangliomas (PPGLs) are rare neuroendocrine tumors derived from chromaffin cells, with 80-85% originating in the adrenal medulla and 15-20% from extra-adrenal chromaffin tissues (paragangliomas). Approximately 30-40% of PPGLs have a hereditary component, making them one of the most genetically predisposed tumor types. Recent advances in genetic research have classified PPGLs into three molecular clusters: pseudohypoxia-related, kinase-signaling, and -signaling pathway variants.
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