A primary right atrium paraganglioma in a 15-year-old patient.
Thoracic pheochromocytomas account for less than 2% of reported cases, while primary cardiac paragangliomas are even rare. The following case illustrates a 15-year-old patient with primary right atrium paraganglioma. This patient was referred for paroxysmal hypertension and excessive perspiration. Pheochromocytoma was suspected and then confirmed by very high serum nor-metanephrine which increased more than 30-fold above the upper limit of normal. 131I-metaiodobenzylguanidine (MIBG) scintigraphy showed high uptake only in the middle mediastinum, but not in the adrenal glands or elsewhere. Both contrast CT and gated MRI of the chest disclosed a 5.0 x 4.0 cm2 mass in the right atrium. Coronary angiography demonstrated the mass with feeding vessels from the right coronary artery. When the patient's blood pressure was well controlled with doxazosin and metoprolol, surgery was then performed. A 6.0 x 4.9 x 4.0 cm3 round solid right atrium paraganglioma weighing 41.7 g was resected. The second day after surgery, serum nor-metanephrine and urinary noradrenaline levels dropped rapidly to normal range, and the patient was free of clinical symptoms with normal BP. Postoperative cardiac function, as measured by echocardiogram, was normal. Although cardiac paraganglioma may be difficult to resect, it can be cured.
Shanghai Clinical Center for Endocrine and Metabolic Diseases, 197 Ruijin Er Lu, Shanghai, 200025, PR China.
Wen-Qi
WQ
Yuan
Wei-Qinq
WQ
Wang
Ting-Wei
TW
Su
Hai-Tao
HT
Chen
Zhong-Wei
ZW
Shi
Wen-Qiang
WQ
Fang
Biao
B
Li
Xiao-Long
XL
Jin
Wang-Fu
WF
Zang
Guang
G
Ning
D000293:Adolescent; D005260:Female; D006325:Heart Atria; D006338:Heart Neoplasms / Q000150:complications / Q000175:diagnosis* / Q000473:pathology; D006801:Humans; D006945:Hyperhidrosis / Q000209:etiology; D006973:Hypertension / Q000209:etiology; D008279:Magnetic Resonance Imaging; D010236:Paraganglioma, Extra-Adrenal / Q000150:complications / Q000175:diagnosis* / Q000473:pathology; D010673:Pheochromocytoma / Q000150:complications / Q000175:diagnosis* / Q000473:pathology