We describe a 26-year-old woman presenting with chest pain and evidence of coronary ischemia. Echocardiography revealed a large left ventricular mass initially deemed unresectable at her initial institution. Investigation revealed a dopamine-secreting primary cardiac paraganglioma encompassing vital cardiac architecture. This case discusses our heart team approach to complex cardiac masses and illustrates the feasibility of surgical resection in complex cases of hormonally active primary cardiac paragangliomas.
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http://dx.doi.org/10.1016/j.athoracsur.2021.05.042 | DOI Listing |
JNCI Cancer Spectr
January 2025
Section on Medical Neuroendocrinology National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, 20892, MD, USA.
Head and neck paragangliomas (HNPGLs) are typically slow-growing, hormonally inactive tumors of parasympathetic paraganglia. Inactivation of prolyl-hydroxylase domain-containing 2 protein causing indirect gain-of-function of hypoxia-inducible factor-2α (HIF-2α), encoded by EPAS1, was recently shown to cause carotid body hyperplasia. We previously described a syndrome with multiple sympathetic paragangliomas caused by direct gain-of-function variants in EPAS1 (Pacak-Zhuang syndrome, PZS) and developed a corresponding mouse model.
View Article and Find Full Text PDFAnn Thorac Surg Short Rep
September 2024
Division of Pediatric Cardiac Surgery, Department of Cardiovascular Surgery, Children's Heart Institute, Children's Memorial Hermann Hospital, University of Texas Health Science Center at Houston McGovern Medical School, Houston, Texas.
Aorticopulmonary paragangliomas are exceedingly rare tumors in pediatric populations. Complete surgical resection is the only curative treatment. However, resection is challenging due to the vascularity of paragangliomas and their close relationship with the great vessels.
View Article and Find Full Text PDFCureus
December 2024
Internal Medicine, Texas Tech University Health Sciences Center, El Paso, USA.
Carotid body tumors (CBTs), rare neuroendocrine neoplasms near the carotid bifurcation, are mostly asymptomatic but may cause discomfort and autonomic dysfunction. Computed tomography angiography (CTA) is used for diagnosis, eliminating the need for a biopsy to avoid the risk of hemorrhage. Surgical excision is the preferred treatment, while radiotherapy is an option when surgery is impractical.
View Article and Find Full Text PDFDiscov 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 PDFCardiol Rev
October 2024
Barbra Streisand Women's Heart Center, Smidt Heart Institute, Cedars Sinai Medical Center, Los Angeles, CA.
Arterial hypertension in young adults, which includes patients between 19 and 40 years of age, has been increasing in recent years and is associated with a significantly higher risk of target organ damage and short-term mortality. It has been reported that up to 10% of these cases are due to a potentially reversible secondary cause, mainly of endocrine (primary aldosteronism, Cushing's syndrome, and pheochromocytoma/paraganglioma), renal (renovascular hypertension due to fibromuscular dysplasia and renal parenchymal disease), or cardiac (coarctation of the aorta) origin. It is recommended to rule out a secondary cause of high blood pressure (BP) in those patients with early onset of grade 2 or 3 hypertension, acute worsening of previously controlled hypertension, resistant hypertension, hypertensive emergency, severe target organ damage disproportionate to the grade of hypertension, or in the face of clinical or biochemical characteristics suggestive of a secondary cause of hypertension.
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