LETTER
Diagnosing Pheochromocytoma in von Hippel-Lindau Disease
A. K. Jain;
S. L. Chew; and
A. Grossman
15 September 1994 | Volume 121 Issue 6 | Pages 465-466
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.
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Author and Article Information
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St. Bartholomew's Hospital, London EC1A 7BE, England
1. Aprill BS, Drake AJ III, Lasseter DH, Shakir KM. Silent adrenal nodules in von Hippel-Lindau disease suggest pheochromocytoma. Ann Intern Med. 1994; 120:485-7.
2. Chew SL, Dacie JE, Reznek RH, Newbould EC, Sheaves R, Trainer PJ, et al. Bilateral pheochromocytomas in von Hippel-Lindau disease: diagnosis by adrenal vein sampling and catecholamine assay. Q J Med. 1994; 87:49-54.
6. Allison DJ, Brown MJ, Jones DH, Timmis JB. Role of venous sampling in locating a pheochromocytoma. Br Med J. 1983; 286:1122-4.
7. Harrison TS, Freier DT. Pitfalls in the technique and interpretation of regional venous sampling for localising pheochromocytoma. Surg Clin North Am. 1974; 54:339-47.
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