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Sporadic Paraganglioma

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Abstract

Paraganglioma are rare chromaffin cell tumors that develop from the neural crest cells. Parasympathetic ganglia-derived tumors are found almost exclusively in the neck and skull base and typically do not secrete catecholamines. In contrast, most sympathetic ganglia-derived tumors are found in the abdomen and produce an excess of catecholamines. Those grow within the adrenal medulla and are known as pheochromocytoma. Other sympathetic paragangliomas are also known as extra-adrenal pheochromocytomas. They arise outside of the adrenal gland and can be found anywhere along the sympathetic chain from the base of the skull and neck (5% of cases) to the bladder and prostate gland (10%). We use the term paraganglioma to refer to extra-adrenal tumors and pheochromocytoma to refer to intra-adrenal tumors. The majority of sympathetic paragangliomas are familial, commonly associated with the SDHB and SDHD gene mutations. Many are malignant. Medical management of catecholamine-secreting paraganglioma is the same as for pheochromocytoma. Favorably located abdominal paragangliomas can be safely resected laparoscopically, but the operation is technically more challenging than that for adrenalectomy.

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References

  1. Erickson D, Kudva YC, Ebersold MJ, et al. (2001) Benign paragangliomas: clinical presentation and treatment outcomes in 236 patients. J Clin Endocrinol Metab 86(11):5210–5216

    Article  PubMed  CAS  Google Scholar 

  2. Whalen RK, Althausen AF, Daniels GH (1992) Extra-adrenal pheochromocytoma. J Urol 147(1):1–10

    PubMed  CAS  Google Scholar 

  3. Young WF Jr (2006) Paragangliomas: clinical overview. Ann N Y Acad Sci 1073:21–29

    Article  PubMed  CAS  Google Scholar 

  4. Elder EE, Elder G, Larsson C (2005) Pheochromocytoma and functional paraganglioma syndrome: no longer the 10% tumor. J Surg Oncol 89(3):193–201

    Article  PubMed  Google Scholar 

  5. Plouin PF, Gimenez-Roqueplo AP (2006) Pheochromocytomas and secreting paragangliomas. Orphanet J Rare Dis 1(1):49

    Article  PubMed  Google Scholar 

  6. Sclafani LM, Woodruff JM, Brennan MF (1990) Extraadrenal retroperitoneal paragangliomas: natural history and response to treatment. Surgery 108(6):1124–1129; discussion 9–30

    PubMed  CAS  Google Scholar 

  7. Gifford RW Jr, Manger WM, Bravo EL (1994) Pheochromocytoma. Endocrinol Metab Clin North Am 23(2):387–404

    PubMed  Google Scholar 

  8. Mantero F, Albiger N (2004) A comprehensive approach to adrenal incidentalomas. Arq Bras Endocrinol Metabol 48(5):583–591

    PubMed  Google Scholar 

  9. Ogawa J, Inoue H, Koide S, et al. (1982) Functioning paraganglioma in the posterior mediastinum. Ann Thorac Surg 33(5):507–510

    Article  PubMed  CAS  Google Scholar 

  10. Plouin PF, Duclos JM, Menard J, et al. (1981) Biochemical tests for diagnosis of phaeochromocytoma: urinary versus plasma determinations. Br Med J (Clin Res Ed) 282(6267):853–854

    CAS  Google Scholar 

  11. Leestma JE, Price EB Jr (1971) Paraganglioma of the urinary bladder. Cancer 28(4):1063–1073

    Article  PubMed  CAS  Google Scholar 

  12. Bravo EL, Tagle R (2003) Pheochromocytoma: state-of-the-art and future prospects. Endocr Rev 24(4):539–553

    Article  PubMed  CAS  Google Scholar 

  13. Bravo EL, Tarazi RC, Gifford RW, et al. (1979) Circulating and urinary catecholamines in pheochromocytoma. Diagnostic and pathophysiologic implications. N Engl J Med 301(13):682–686

    Article  PubMed  CAS  Google Scholar 

  14. Kudva YC, Sawka AM, Young WF Jr (2003) Clinical review 164: The laboratory diagnosis of adrenal pheochromocytoma: the Mayo Clinic experience. J Clin Endocrinol Metab 88(10):4533–4539

    Article  PubMed  CAS  Google Scholar 

  15. Sawka AM, Jaeschke R, Singh RJ, et al. (2003) A comparison of biochemical tests for pheochromocytoma: measurement of fractionated plasma metanephrines compared with the combination of 24-hour urinary metanephrines and catecholamines. J Clin Endocrinol Metab 88(2):553–558

    Article  PubMed  CAS  Google Scholar 

  16. Lenders JW, Eisenhofer G, Armando I, et al. (1993) Determination of metanephrines in plasma by liquid chromatography with electrochemical detection. Clin Chem 39(1):97–103

    PubMed  CAS  Google Scholar 

  17. Lenders JW, Keiser HR, Goldstein DS, et al. (1995) Plasma metanephrines in the diagnosis of pheochromocytoma. Ann Intern Med 123(2):101–109

    PubMed  CAS  Google Scholar 

  18. Lee JA Zarnegar R, Shen WT, et al. (2007) Adrenal incidentaloma, borderline elevations of urine or plasma metanephrines and the “subclinical” pheochromocytoma. Arch Surg 142(9):870-873; discussion 73-74

    Article  Google Scholar 

  19. Hsiao RJ, Parmer RJ, Takiyyuddin MA, et al. (1991) Chromogranin A storage and secretion: sensitivity and specificity for the diagnosis of pheochromocytoma. Medicine 70(1):33–45

    Article  PubMed  CAS  Google Scholar 

  20. Young WF Jr (1997) Phaeochromocytoma: how to catch a moonbeam in your hand. Eur J Endocrinol 136(1):28–29

    Article  PubMed  CAS  Google Scholar 

  21. Manger WM, Eisenhofer G (2004) Pheochromocytoma: diagnosis and management update. Curr Hypertens Rep 6(6):477–484

    Article  PubMed  Google Scholar 

  22. Pacak K, Eisenhofer G, Ilias I (2004) Diagnostic imaging of pheochromocytoma. Front Horm Res 31:107–120

    PubMed  Google Scholar 

  23. Quint LE, Glazer GM, Francis IR, et al. (1987) Pheochromocytoma and paraganglioma: comparison of MR imaging with CT and I-131 MIBG scintigraphy. Radiology 165(1):89–93

    PubMed  CAS  Google Scholar 

  24. Bessell-Browne R, O’Malley ME (2007) CT of pheochromocytoma and paraganglioma: risk of adverse events with i.v. administration of nonionic contrast material. AJR Am J Roentgenol 188(4):970–974

    Article  PubMed  Google Scholar 

  25. Mukherjee JJ, Peppercorn PD, Reznek RH, et al. (1997) Pheochromocytoma: effect of nonionic contrast medium in CT on circulating catecholamine levels. Radiology 202(1):227–231

    PubMed  Google Scholar 

  26. Francis IR, Korobkin M (1996) Pheochromocytoma. Radiol Clin North Am 4(6):1101–1112

    Google Scholar 

  27. Honigschnabl S, Gallo S, Niederle B, et al. (2002) How accurate is MR imaging in characterisation of adrenal masses: update of a long-term study. Eur J Radiol 41(2):113–122

    Article  PubMed  Google Scholar 

  28. Timmers HJ, Kozupa A, Chen CC, et al. (2007) Superiority of fluorodeoxyglucose positron emission tomography to other functional imaging techniques in the evaluation of metastatic SDHB-associated pheochromocytoma and paraganglioma. J Clin Oncol 25(16):2262–2269

    Article  PubMed  Google Scholar 

  29. Ilias I, Pacak K (2004) Current approaches and recommended algorithm for the diagnostic localization of pheochromocytoma. J Clin Endocrinol Metab 89(2):479–491

    Article  PubMed  CAS  Google Scholar 

  30. Elgazzar AH, Gelfand MJ, Washburn LC, et al. (1995) I-123 MIBG scintigraphy in adults. A report of clinical experience. Clin Nucl Med 20(2):147–152

    Article  PubMed  CAS  Google Scholar 

  31. Pacak K, Eisenhofer G, Ahlman H, et al. (2007) Pheochromocytoma: recommendations for clinical practice from the First International Symposium, October 2005. Nat Clin Pract Endocrinol Metab 3(2):92–102

    Article  PubMed  Google Scholar 

  32. Grufferman S, Gillman MW, Pasternak LR, et al. (1980) Familial carotid body tumors: case report and epidemiologic review. Cancer 46(9):2116–2122

    Article  PubMed  CAS  Google Scholar 

  33. Amar L, Bertherat J, Baudin E, et al. (2005) Genetic testing in pheochromocytoma or functional paraganglioma. J Clin Oncol 23(34):8812–8818

    Article  PubMed  CAS  Google Scholar 

  34. Neumann HP, Bausch B, McWhinney SR, et al. (2002) Germ-line mutations in nonsyndromic pheochromocytoma. N Engl J Med 346(19):1459–1466

    Article  PubMed  CAS  Google Scholar 

  35. Neumann HP, Pawlu C, Peczkowska M, et al. (2004) Distinct clinical features of paraganglioma syndromes associated with SDHB and SDHD gene mutations. JAMA 292(8):943–951

    Article  PubMed  CAS  Google Scholar 

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Authors and Affiliations

  1. Department of Surgery, Columbia University Medical Center, 161 Fort Washington Avenue, New York, New York, 10032, USA

    James A. Lee

  2. Surgical Service, VA Medical Center, 4150 Clement Street, San Francisco, California, 94121, USA

    Quan-Yang Duh

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  1. James A. Lee
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  2. Quan-Yang Duh
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Correspondence to Quan-Yang Duh.

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Lee, J.A., Duh, QY. Sporadic Paraganglioma. World J Surg 32, 683–687 (2008). https://doi.org/10.1007/s00268-007-9360-4

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  • DOI: https://doi.org/10.1007/s00268-007-9360-4

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