Diagnosing Pheochromocytoma in von Hippel-Lindau Disease
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TO THE EDITOR:
Aprill and colleagues [1] report valuable data on small adrenal nodules in patients with von Hippel-Lindau disease. Their patients show the difficulties of a preoperative diagnosis of pheochromocytoma when the adrenal lesions are so small. However, the use of adrenal vein sampling and a catecholamine assay might have helped. The St. Bartholomew's Hospital experience in four unrelated patients with von Hippel-Lindau disease shows its value [2]. In this series, adrenal vein noradrenaline-to-adrenaline ratios were measured in patients with small adrenal masses that had been found on CT scans. Radiolabeled metaiodo-benzylguanidine (MIBG) and T2-weighted magnetic resonance imaging (MRI) had not been helpful in determining whether these masses were pheochromocytomas. Every adrenal gland with histologically confirmed pheochromocytoma had an adrenal vein adrenaline-to-noradrenaline ratio greater than 1. Adrenal glands with nonpheochromocytoma lesions (eight patients with the Conn syndrome were used as controls) had adrenal vein noradrenaline-to-adrenaline ratios between 0.06 and 0.6.
Venous sampling may have been valuable in the first and fourth patients described by Aprill and colleagues but less helpful in patients 2 and 3, who had other findings suggesting pheochromocytoma (positive MIBG scintigraphy or a high T2-weighted signal on MRI). Although well-documented [2-4], the hazards of venous sampling are minimal in experienced hands, and its benefits exceed the risks.
The Editors welcome submissions for possible publication in the Letters section. Authors of letters should:
•Include no more than 300 words of text, three authors, and five references
•Type with double-spacing
•Send three copies of the letter, an authors' form signed by all authors, and a cover letter describing any conflicts of interest related to the contents of the letter.
Letters commenting on an Annals article will be considered if they are received within 6 weeks of the time the article was published. Only some of the letters received can be published. Published letters are edited and may be shortened; tables and figures are included only selectively. Authors will be notified that the letter has been received. If the letter is selected for publication, the author will be notified about 3 weeks before the publication date. Unpublished letters cannot be returned.
Annals welcomes electronically submitted letters.
- Copyright ©2004 by the American College of Physicians
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