A SERIES OF TWO PATIENTS WITH CARDIAC PARAGANGLIOMAS

AACE Clinical Case Reports
Hannah E FarquharAshim Sinha

Abstract

The objective of this report is to present 2 cases of cardiac paragangliomas (PGLs), and to outline the presentation, management, and associated genetic mutations. Case 1, a 38-year-old female, presented with a 12-month history of paroxysmal palpitations, headaches, and weight loss. Her investigations included plasma free metanephrines and urinary metanephrines, 68-gallium DOTATATE positron emission tomography/computed tomography, and cardiac imaging. Case 2, a 28-year-old male, presented with a hypertensive crisis and abdominal pain on a background of hypertension. Given his abdominal pain, he was investigated with an abdominal computed tomography (CT) scan, followed by plasma free meta-nephrines and urinary metanephrines, echocardiogram, and 123-iodine meta-iodobenzylguanidine single-photon emission CT. Case 1 had an elevated plasma normetadrenaline of 6,750 pmol/L (reference range is <900 pmol/L) and 3-methoxytyramine of 1,845 pmol/L (reference range is <110 pmol/L). 68-gallium DOTATATE positron emission tomography/computed tomography showed an avid cardiac lesion. The lesion was resected, and histopathology confirmed PGL. Genetic studies revealed an SDHC gene mutation. For case 2, abdominal CT revealed a para-spinal mass. W...Continue Reading

References

Jan 29, 2008·World Journal of Surgery·James A Lee, Quan-Yang Duh
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May 1, 2012·The Annals of Thoracic Surgery·Lachlan MarshallJulie Mundy
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Oct 22, 2014·Journal of Cardiac Surgery·Ji-Gang WangYu-Jun Li
Nov 8, 2018·The Journal of Thoracic and Cardiovascular Surgery·Xin Pei LiuJian Zhou Liu

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Citations

Oct 9, 2021·Journal of Nuclear Cardiology : Official Publication of the American Society of Nuclear Cardiology·Maria E MarketouSophia Koukouraki

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