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Ectopic pheochromocytoma: Does the rule of tens apply?

Lookup NU author(s): Richard Bliss, Professor Thomas Lennard


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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.

Publication metadata

Author(s): Madani R, Al-Hashmi M, Bliss R, Lennard TWJ

Publication type: Article

Publication status: Unknown

Journal: World Journal of Surgery

Year: 2007

Volume: 31

Issue: 4

Pages: 849-854

ISSN (print): 0364-2313

ISSN (electronic): 1432-2323


DOI: 10.1007/s00268-006-0608-1


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