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



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.


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


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.


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


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.


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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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