A primary right atrium paraganglioma in a 15-year-old patient

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• 作者：Wen-Qi Yuan (1)
  Wei-Qinq Wang (1)
  Ting-Wei Su (1)
  Hai-Tao Chen (2)
  Zhong-Wei Shi (3)
  Wen-Qiang Fang (4)
  Biao Li (5)
  Xiao-Long Jin (6)
  Wang-Fu Zang (2)
  Guang Ning (1)
  
• 关键词：Paraganglioma ; Right atrium ; Heart ; Pheochromocytoma
• 刊名：Endocrine
• 出版年：2007
• 出版时间：October 2007
• 年：2007
• 卷：32
• 期：2
• 页码：245-248
• 全文大小：254KB
• 参考文献：1. S.A. Lagerstedt, D.J. O’Kane, R.J. Singh, Clin. Chem. 50, 603-11 (2004) CrossRef
  2. J.T. Andrew, H. Steve, J.S. Michael, Eur. J. Cardiothorac. Surg. 28, 352-54 (2005) CrossRef
  3. K. Hiroyuki, Y. Tamotsu, N. Hiroshi, et?al., Jpn. Circ. J. 65, 315-19 (2001) CrossRef
  4. R.W. Gifford Jr., W.M. Manger, E.I. Bravo, Endocrinol. Metab. 23, 387-04 (1994)
  5. E. Brown, M. Milshteyn, B. Kleinman, et?al., J. Cardiothorac. Vasc. Anesth. 16, 370-73 (2002) CrossRef
  6. J. Valluvan, C. Mehmet, S. Brahm, et?al., Ann. Surg. 4, 415-19 (1995)
  7. P.J. Fitzgerald, T.A. Ports, M.D. Cheitlin, et?al., Cardiovasc. Surg. 3, 557-61 (1995) CrossRef
  8. T. Gomi, T. Ikeda, J. Sakurai, et?al., Jpn. Heart J. 35, 117-24 (1994)
  9. A. Jonsson, B. Hallengren, P. Manhem, et?al., J. Intern. Med. 236, 93-6 (1994) CrossRef
  10. H.L. Hongpyo, I.B. William, V. Indubala, et?al., Chest 97, 760-62 (1990) CrossRef
  11. V.A. Jebara, M.S. Uva, A. Farge, et?al., Ann. Thorac. Surg. 53, 356-61 (1992) CrossRef
  12. J. Lin, B. Palafox, H. Jackson, et?al., Ann. Thorac. Surg. 67, 555-58 (1999) CrossRef
  
• 作者单位：Wen-Qi Yuan (1)
  Wei-Qinq Wang (1)
  Ting-Wei Su (1)
  Hai-Tao Chen (2)
  Zhong-Wei Shi (3)
  Wen-Qiang Fang (4)
  Biao Li (5)
  Xiao-Long Jin (6)
  Wang-Fu Zang (2)
  Guang Ning (1)
  
  1. Shanghai Clinical Center for Endocrine and Metabolic Diseases, Shanghai Institute of Endocrinology and Metabolism, Shanghai Key Laboratory for Endocrine Tumors, Division of Endocrine and Metabolic Diseases of E-Institute of Shanghai Universities, RuiJin Hospital, Shanghai JiaoTong University School of Medicine, 197 Ruijin Er Lu, Shanghai, 200025, P.R. China
  2. Department of Cardiothoracic Surgery, Ruijin Hospital, Jiaotong University School of Medicine, Shanghai, China
  3. Department of Cardiology, Ruijin Hospital, Jiaotong University School of Medicine, Shanghai, China
  4. Department of Radiology, Ruijin Hospital, Jiaotong University School of Medicine, Shanghai, China
  5. Department of Nuclear Medicine, Ruijin Hospital, Jiaotong University School of Medicine, Shanghai, China
  6. Department of Pathology, Ruijin Hospital, Jiaotong University School of Medicine, Shanghai, China
  

文摘

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?×?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?×?4.9?×?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.