Annals
Established in 1927 by the American College of Physicians
:
Advanced search
 
box Article
 arrow  Table of Contents                
space
box Services
 arrow  Send comment/rapid response letter
space
 arrow  Notify a friend about this article
space
 arrow  Alert me when this article is cited
space
 arrow  Add to Personal Archive
space
 arrow  Download to Citation Manager
space
 arrow  ACP Search                        
space
 arrow  Get Permissions
space
box Google Scholar
 arrow  Search for Related Content
space
box PubMed
Articles in PubMed by Author:
  arrow  Aprill, B.
space
  arrow  Shakir, K. M. M.
space
 arrow  PubMed                        
space

REPLY

Diagnosing Pheochromocytoma in von Hippel-Lindau Disease

right arrow Brian Aprill; Almond J. Drake; and K. M. Mohamed Shakir

15 September 1994 | Volume 121 Issue 6 | Pages 465-466


IN RESPONSE:

We concur with Dr. Siddiqui's comments that CT scanning is highly sensitive in visualizing intra-adrenal pheochromocytomas [1]. However, CT scanning alone is not sufficient because of its relatively low specificity in distinguishing pheochromocytomas from other adrenal masses such as adrenal incidentalomas [1, 2]. Although MIBG scanning and MRI with T2-weighted images may be more specific for pheochromocytoma [1], diagnosis rests mainly on a high clinical suspicion (particularly in familial syndromes such as von Hippel-Lindau disease) and appropriate biochemical testing.

The disparity in the surgical specimen and CT scan sizes of the left adrenal mass in patient 1 was caused by several factors. A re-evaluation of the original CT scan showed the nodule to be closer to 1.5 cm in greatest diameter. The fixed and sectioned adrenal mass was 1 cm in total diameter, in contrast to 2 cm as noted in the operative report. It was composed of an irregular, nonencapsulated 0.5-cm internal pheochromocytoma component surrounded by a rim of normal adrenal cortical tissue. The slight difference in the total diameter of the mass as measured by CT scanning compared with histologic measurement was probably due to fixation artifact and shrinkage. Although we agree that the "typical" 3- to 5-cm pheochromocytoma is generally encapsulated [3], our experience and that of others show that small, clinically silent lesions may not be encapsulated (Ogorzalek JM. Personal communication).

The CT scan of the abdomen in patient 2 showed a 3.5-cm left adrenal nodule and a normal right adrenal gland. Bilateral adrenalectomy (done after bilateral positive uptake on MIBG scanning) showed a 4.0-cm left adrenal mass and a 2.2-cm right adrenal mass that were both encapsulated and salmon-colored, with adherent rims of residual yellow adrenal cortical tissue. Both masses were histologically confirmed to be pheochromocytomas. Of interest, the CT scan did not show the right adrenal mass. None of our patients have shown any evidence of malignancy.

We agree with Jain and colleagues' suggestion that venous sampling and determination of the adrenal vein noradrenaline-to-adrenaline ratio are valuable [4] for confirming an intra-adrenal pheochromocytoma. However, three of the four patients described by Chew and colleagues [4] had clearly elevated urinary noradrenaline excretion, and venous sampling showed a contralateral second tumor in each. Thus, venous sampling is effective in locating small bilateral pheochromocytomas in patients with elevated 24-hour urinary norepinephrine levels. Our patient 1 had normal urinary and plasma catecholamine values, so we do not know if venous sampling would have shown an intra-adrenal pheochromocytoma. Even in patient 3, venous sampling may not have disclosed a pheochromocytoma at the initial evaluation when the patient was normotensive and had normal biochemical test results. Further studies of adrenal venous sampling are necessary to answer this question.


Author and Article Information
space
up arrowTop
dotAuthor & Article Info
down arrowReferences

Naval Medical Center, Oakland, CA 94627. National Naval Medical Center, Bethesda, MD 20889-5600.
Disclaimer: The opinions and assertions contained herein are those of the authors and are not to be construed as official or as reflecting the views of the Navy Department or the Naval Service at large.


References
space
up arrowTop
up arrowAuthor & Article Info
dotReferences

1. Stein PP, Black HR. A simplified diagnostic approach to pheochromocytoma. Medicine (Balt). 1990; 70:46-66.

2. Gross MD, Shapiro B. Clinically silent adrenal masses. J Clin Endocrinol Metab. 1993; 77:885-8.

3. Sternberg SS, ed. Diagnostic Surgical Pathology. New York: Raven Press; 1989:461-3.

4. 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.

About Letters
space

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.





box Article
 arrow  Table of Contents                
space
box Services
 arrow  Send comment/rapid response letter
space
 arrow  Notify a friend about this article
space
 arrow  Alert me when this article is cited
space
 arrow  Add to Personal Archive
space
 arrow  Download to Citation Manager
space
 arrow  ACP Search                        
space
 arrow  Get Permissions
space
box Google Scholar
 arrow  Search for Related Content
space
box PubMed
Articles in PubMed by Author:
  arrow  Aprill, B.
space
  arrow  Shakir, K. M. M.
space
 arrow  PubMed                        
space


 Home | Current Issue | Past Issues | In the Clinic | ACP Journal Club | CME | Collections | Audio/Video | Mobile | Subscribe | Tools | Help | ACP Online