The Korean Journal of Medicine

The Korean Association of Internal Medicine (대한내과학회)
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Diagnosis and Treatment of Pheochromocytoma

갈색세포종의 진단과 치료

  • Yoo, Soon-Jib (Division of Endocrinology and Metabolism, Department of Internal Medicine, The Catholic University of Korea College of Medicine, Bucheon St. Mary's Hospital)
  • 유순집 (가톨릭대학교 의과대학 부천성모병원 내분비내과)
  • Published : 2012.04.01
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Abstract

Pheochromocytomas are rare neuroendocrine tumours with a highly variable and heterogeneous clinical manifestation. With a noticeable progress in genetics, biochemical diagnosis and tumour imaging techniques, modifications of the traditional "rule of tens" for pheochromocytomas are inevitable consequence. Diagnosis of pheochromocytoma depends on biochemical evidence of catecholamine production by the tumor. Plasma free metanephrines provide the best test for excluding or confirming pheochromocytoma and should be used as an initial screening tool, but if it is not available, urinary fractionated metanephrines could be used as an alternative method. Different anatomical and functional imaging modalities are used to localize biochemically proven pheochromocytomas. These include computed tomography, magnetic resonance imaging, single-photon emission computed tomography (SPECT) using $^{123/131}I$-metaiodobenzylguanidine (MIBG) or $^{111}In$-DTPA-pentetreotide and positron emission tomography (PET). Timely laparoscopic tumour removal in combination with appropriate pre-and intraoperative management of the effects of secreted catecholamines are essential components for excellent prognosis.

Keywords

References

  1. Pacak K, Timmers H J.L.M., Eisenhofer G. Pheochromoyotma. In: Jameson JL, De Groot LJ, eds. Endocrinology. 6th ed. Philadelphia: Saunders Elsevier, 2010:1990-2018.
  2. William FY Jr. Endocrine hypertension. In: Melmed S, Polonsky KS, Larsen PR, Kronenberg HM, eds. Williams Textbook of Endocrinology. 12th ed. Philadelphia: Elsevier/Saunders, 2011:545-577.
  3. Neumann HPH. Pheochromocytoma. In: Longo DL, Fauci A, Kasper DL, Hauser SL, Jameson JL, Loscalzo J, eds. Harrison's Principles of Internal Medicine. 18th ed. McGrow Hill, 2011:2962-2967.
  4. Song YD. Adrenal cortex and endocrine hypertension. In: Korean Endocrin Society, ed. Texbook of endocrinology and metabolism. 2nd ed. Seoul: Koonja Publishing, 2011:342-375.
  5. Young WF Jr. Adrenal causes of hypertension: pheochromocytoma and primary aldosteronism. Rev Endocr Metab Disord 2007;8:309-320. https://doi.org/10.1007/s11154-007-9055-z
  6. Karagiannis A, Mikhailidis DP, Athyros VG, Harsoulis F. Pheochromocytoma: an update on genetics and management. Endocr Relat Cancer 2007;14:935-956. https://doi.org/10.1677/ERC-07-0142
  7. Lenders JW, Pacak K, Walther MM, et al. Biochemical diagnosis of pheochromocytoma: which test is best? JAMA 2002;287:1427-1434. https://doi.org/10.1001/jama.287.11.1427
  8. Lenders JW, Eisenhofer G, Mannelli M, Pacak K. Phaeochromocytoma. Lancet 2005;366:665-675. https://doi.org/10.1016/S0140-6736(05)67139-5
  9. Eisenhofer G. Screening for pheochromocytomas and paragangliomas. Curr Hypertens Rep 2012;14:130-137. https://doi.org/10.1007/s11906-012-0246-y
  10. Blake MA, Cronin CG, Boland GW. Adrenal imaging. AJR Am J Roentgenol 2010;194:1450-1460. https://doi.org/10.2214/AJR.10.4547
  11. Choi CH, Kim DY, Kim JS, Cho BY. Neuroendocrine tumor. In: Chung JK, Lee MC, eds. Nuclear Medicine. 3rd ed. Seoul: Korea Medical Book Publisher, 2008:497-509.
  12. Pacak K, Eisenhofer G, Goldstein DS. Functional imaging of endocrine tumors: role of positron emission tomography. Endocr Rev 2004;25:568-580. https://doi.org/10.1210/er.2003-0032
  13. Pacak K, Eisenhofer G, Ahlman H, et al; International Symposium on Pheochromocytoma. Pheochromocytoma: Recommendations for clinical practice from the First International Symposium. October 2005. Nat Clin Pract Endocrinol Metab 2007;3:92-102. https://doi.org/10.1038/ncpendmet0396
  14. Kasturi S, Kutikov A, Guzzo TJ, Smith AL, Wein AJ. Modern management of pheochromocytoma. Nat Clin Pract Urol 2007;4:630-633. https://doi.org/10.1038/ncpuro0962
  15. Zeiger MA, Siegelman SS, Hamrahian AH. Medical and surgical evaluation and treatment of adrenal incidentalomas. J Clin Endocrinol Metab 2011;96:2004-2015. https://doi.org/10.1210/jc.2011-0085
  16. Scholz T, Eisenhofer G, Pacak K, Dralle H, Lehnert H. Clinical review: Current treatment of malignant pheochromocytoma. J Clin Endocrinol Metab 2007;92:1217-1225. https://doi.org/10.1210/jc.2006-1544

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