[Skip to Content]
Access to paid content on this site is currently suspended due to excessive activity being detected from your IP address 54.159.189.139. Please contact the publisher to request reinstatement.
[Skip to Content Landing]
Citations 0
Letters
February 8, 2006

Paraganglioma Syndrome and Mutations of the

Author Affiliations
 

Letters Section Editor: Robert M. Golub, MD, Senior Editor.

JAMA. 2006;295(6):625. doi:10.1001/jama.295.6.625-b

In Reply: We thank Dr Niemann for pointing out the citation errors in our tables. The definitions of malignant disease for pheochromocytoma and paraganglioma remain controversial; given limited technology, they are operational definitions. Over the course of decades, experts have not come up with a consensus. For our study, we chose to use the most conservative operational definition, ie, only patients with distant metastases. The only absolute criterion for diagnosing a malignant pheochromocytoma is the presence of secondary tumor deposits in sites where chromaffin tissue is not normally found. This eliminates tumors of multicentric origin, which are usually benign and amenable to surgery.1 We do not believe that our conclusions are affected by our review of the literature.

First Page Preview View Large
First page PDF preview
First page PDF preview
×