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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.

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