, Volume 31, Issue 4, pp 849-854
Date: 09 Mar 2007

Ectopic Pheochromocytoma: Does the Rule of Tens Apply?

Rent the article at a discount

Rent now

* Final gross prices may vary according to local VAT.

Get Access

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

An erratum to this article is available at http://dx.doi.org/10.1007/s00268-007-9251-8.