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World Journal of Surgery

, Volume 31, Issue 4, pp 849–854 | Cite as

Ectopic Pheochromocytoma: Does the Rule of Tens Apply?

  • R. Madani
  • M. Al-Hashmi
  • R. Bliss
  • T.W.J. LennardEmail author
Article

Abstract

Introduction

The rule of “tens” is often stated to reflect the distribution and histology of pheochromocytomas, with 10% being bilateral, 10%; ectopic in origin, and 10%; malignant. The objective of this study was to review the ectopic pheochromocytomas in a tertiary endocrine center and to establish whether the rule of tens holds true.

Methods

Retrospective data were collected on all adrenalectomies and ectopic pheochromocytoma resections performed between 1993 and 2005 at our institution.

Results

In total, 77 patients had pheochromocytomas: 75%; (58/77) adrenal and 25%; (19/77) ectopic. Of the adrenal pheochromocytomas, 10%; (6/58) were bilateral. The anatomic locations of the ectopic pheochromocytomas were as follows: 26%; (5/19) adjacent to the adrenals, 53%; (10/19) in the organ of Zuckerkandl, 11%; (2/19) in the bladder, 5%; (1/19) in the mediastinum, and 5%;(1/19) in the neck.

Conclusions

Our series demonstrates an incidence of 10%; for bilateral pheochromocytoma, which is similar to that in the published reports. However, 25%; of the pheochromocytomas were ectopic. Zuckerkandl pheochromocytomas were the most common among the ectopic lesions. Rarely, these tumors present outside the abdominal cavity

Keywords

Paragangliomas Metanephrine Adrenal Pheochromocytoma Chromic Salt Multiple Endocrine Neoplasia Syndrome 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

References

  1. 1.
    Sandur S, Dasgupta A, Shapiro JL, et al. Thoracic involvement with pheochromocytoma. Chest 1999;115:511–521PubMedCrossRefGoogle Scholar
  2. 2.
    Manager WM, Gifford RW, Hoffman BB. Pheochromocytoma: a clinical and experimental overview. Curr Probl Cancer 1985;9:1–89Google Scholar
  3. 3.
    Beard CM, Sheps SG, Kurtland LT. Occurrence of pheochromocytoma in Rochester, Minnesota, 1950 through 1979. Mayo Clin Proc 1983;58:802–804PubMedGoogle Scholar
  4. 4.
    Sutton MG, Sheps SG, Lie JT. Prevalence of clinically unsuspected pheochromocytoma: review of a 50 year old autopsy series. Mayo Clin Proc 1981;56:354–360PubMedGoogle Scholar
  5. 5.
    Werbel SS, Ober P. Pheochromocytoma, update on diagnosis, localization and management. Med Clin North Am 1995;79:131–153PubMedGoogle Scholar
  6. 6.
    Neumann HPH, Berger DP, Sigmund G. Pheochromocytomas, multiple endocrine neoplasia type 2 and von Hipple-Lindau disease. N Engl J Med 1993;329:1531–1532PubMedCrossRefGoogle Scholar
  7. 7.
    Gimm O, Armanios M, Dziema H, et al. Somatic and occult germ-line mutations in SDHD, a mitochondrial complex II gene, in non familial pheochromocytoma. Cancer Res 2000;60:6822–6825PubMedGoogle Scholar
  8. 8.
    Atiyeh BA, Barakat AJ, Abumrad NN. Extra-adrenal pheochromocytoma. J Nephrol 1997;10:25–29PubMedGoogle Scholar
  9. 9.
    Mahoney EM, Crocker DW, Friend DG, et al. Adrenal and extra adrenal pheochromocytomas: localization by vena cava sampling and observations on renal juxtaglomerular apparatus. J Urol 1972;108:4–8PubMedGoogle Scholar
  10. 10.
    Van Heerden JA, Sheps SG, Hamberger B, et al. Pheochromocytoma: current status and changing trends. Surgery 1982;91:367–373PubMedGoogle Scholar
  11. 11.
    Buckley KM, Whitman GJ, Chew FS. Diaphragmatic pheochromocytoma. AJR Am J Roentgenol 1995;165:260PubMedGoogle Scholar
  12. 12.
    Lenders JW, Pacak K, Walther MM, et al. Biochemical diagnosis of pheochromocytoma: which test is best? JAMA 2002;287:1427–1434PubMedCrossRefGoogle Scholar
  13. 13.
    Peaston R, Lennard TJW, Lai LC. Overnight excretion of urinary catecholamines and metabolites in the detection of pheochromocytoma. J Clin Endocrinol Metab 1996;81:1378–1384PubMedCrossRefGoogle Scholar
  14. 14.
    Whalen RK, Althausen AF, Daniels GH. Extra-adrenal pheochromocytoma. J Urol 1992;147:1–10PubMedGoogle Scholar
  15. 15.
    Goldfarb DA, Novick AC, Bravo EL, et al. Experience with extra-adrenal pheochromocytoma. J Urol 1989;142:931–936PubMedGoogle Scholar
  16. 16.
    Cone TE. Recurrent pheochromocytoma: report of a case in previously treated child. Pediatrics 1958;21:994–999PubMedGoogle Scholar
  17. 17.
    McNicol AM. Differential diagnosis of pheochromocytomas and paragangliomas. Endocr Pathol 2001;12:407–415CrossRefGoogle Scholar
  18. 18.
    Schwartz EL, Mao P, Hernried O, et al. Catecholamine-secreting paraganglioma: the problem of classification. Arch Intern Med 1975;135:978–985PubMedCrossRefGoogle Scholar
  19. 19.
    Stackpole RH, Melicow MM, Uson AC. Pheochromocytoma in children: report of cases and review of the first 100 published cases with follow-up studies. J Pediatr 1992;63:315–330Google Scholar
  20. 20.
    Das S, Bulusu NV, Lowe P. Primary vesical pheochromocytoma. Urology 1983;21:20–25PubMedCrossRefGoogle Scholar
  21. 21.
    Voci V, Olson H, Beilin L. A malignant primary cardiac pheochromocytoma. Surg Rounds 1982;9:88–90Google Scholar
  22. 22.
    Fries JG, Chamberlin JA. Extra-adrenal pheochromocytoma: literature review and report of a cervical pheochromocytoma. Surgery 1968;63:268–275Google Scholar
  23. 23.
    Nagant de Deuchaisnes C, Fanconi A, Alberto P, et al. Pheochromocytoma extra-surrenaliens multiples avec “dystrophie d’Albright” et hamangiomes cutanes. Schweiz Med Wochenschr 1960;33:886–895Google Scholar
  24. 24.
    Shapiro B, Sisson J, Kalff V, et al. The location of middle mediastinal pheochromocytomas. J Thorac Cardiovasc Surg 1984;87:814–820PubMedGoogle Scholar
  25. 25.
    Von Euler US. Increased urinary excretion of noradrenaline and adrenaline in cases of pheochromocytoma. Ann Surg 1951;134:929–933CrossRefGoogle Scholar
  26. 26.
    Von Euler US, Strom G. Present status of diagnosis and treatment of pheochromocytoma. Circulation 1957;15:5–13Google Scholar
  27. 27.
    Dequattro V, Myers M, Campese VM. Pheochromocytoma: diagnosis and therapy. In DeGroot LJ, ed. Endocrinology, 2nd edition. Philadelphia: Saunders; 1989. p. 1780–1797Google Scholar
  28. 28.
    Donohue JP. Diagnosis and management of adrenal tumor. In Skinner DG, Lieskovsky G, (eds). Diagnosis and Management of Genitourinary Cancer. Philadelphia: Saunders; 1988. p. 372–389Google Scholar
  29. 29.
    Lack EE, Cubilla AL, Woodruff JM, et al. Extra-adrenal paragangliomas of retroperitoneum: a clinico-pathologic study of 12 tumors. Am J Surg Pathol 1980;4:105–120Google Scholar
  30. 30.
    Robbins SL, Cotran RS, Kumar V. Robbins’ Pathologic Basis of Disease, 4th edition. Philadelphia: Saunders; 1989. p. 1262Google Scholar
  31. 31.
    Pommier RF, Vetto JT, Billingsly K, et al. Comparison of adrenal and extra-adrenal pheochromocytomas. Surgery 1993;114:1160–1166PubMedGoogle Scholar
  32. 32.
    Crout JR, Sjoerdsma A. Catecholamines in localization of pheochromocytoma. Circulation 1960;22:516–525.PubMedGoogle Scholar

Copyright information

© Société Internationale de Chirurgie 2007

Authors and Affiliations

  • R. Madani
    • 1
    • 2
  • M. Al-Hashmi
    • 1
  • R. Bliss
    • 1
  • T.W.J. Lennard
    • 1
    Email author
  1. 1.Department of SurgeryRoyal Victoria InfirmaryNewcastle upon TyneUK
  2. 2.Department of SurgeryWhittington HospitalLondonUK

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