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DOI:10.2214/AJR.06.0521
AJR 2006; 187:S467-S469
© American Roentgen Ray Society

Imaging of Pheochromocytoma and Incidental Adrenal Lesions: Self-Assessment Module

Felix S. Chew1 and Kevin P. Banks2

1 Department of Radiology, University of Washington, Box 354755, 4245 Roosevelt Way NE, Seattle, WA 98105.
2 Department of Radiology, Brooke Army Medical Center, San Antonio, TX.

Received April 13, 2006; accepted after revision April 13, 2006.

 
The opinions and assertions contained herein are the private views of the authors and are not to be construed as official or as representing the views of the Department of the Army, the Department of the Air Force, or the Department of Defense.

Address correspondence to F. S. Chew (fchew{at}u.washington.edu).


Abstract
Top
Abstract
INTRODUCTION
EDUCATIONAL OBJECTIVES
REQUIRED READING
INSTRUCTIONS
References
 
The educational objectives for this self-assessment module on imaging of pheochromocytoma and incidental adrenal lesions are for the participant to exercise, self-assess, and improve his or her knowledge of the imaging evaluation of pheochromocytoma and incidental adrenal lesions.

Keywords: adrenal gland • genitourinary imaging • kidney • pheochromocytoma


INTRODUCTION
Top
Abstract
INTRODUCTION
EDUCATIONAL OBJECTIVES
REQUIRED READING
INSTRUCTIONS
References
 
This self-assessment module on imaging of pheochromocytoma and incidental adrenal lesions has an educational component and a self-assessment component. The educational component consists of four required articles that the participant should read. The self-assessment component consists of six multiple-choice questions with solutions. All of these materials are available on the ARRS Web site (www.arrs.org). To claim CME and SAM credit, each participant must enter his or her responses to the questions online.


EDUCATIONAL OBJECTIVES
Top
Abstract
INTRODUCTION
EDUCATIONAL OBJECTIVES
REQUIRED READING
INSTRUCTIONS
References
 
By completing this educational activity, the participant will:

  1. Exercise, self-assess, and improve his or her experience in the imaging evaluation of pheochromocytoma.
  2. Exercise, self-assess, and improve his or her information related to the imaging evaluation of incidentally discovered adrenal lesions.


REQUIRED READING
Top
Abstract
INTRODUCTION
EDUCATIONAL OBJECTIVES
REQUIRED READING
INSTRUCTIONS
References
 
(available online at www.arrs.org)

  1. Colby GW, Banks KP, Torres E. AJR teaching file. Incidental adrenal mass and hypertension. AJR2006 ; 187 [suppl]:S470 -S472[Free Full Text]
  2. Elsayes KM, Narra VR, Leyendecker JR, Francis IR, Lewis JS Jr, Brown JJ. MRI of adrenal and extraadrenal pheochromocytoma. AJR 2005;184 :860-867[Free Full Text]
  3. Blake MA, Krishnamoorthy SK, Boland GW, et al. Low-density pheochromocytoma on CT: a mimicker of adrenal adenoma. AJR 2003;181 :1663-1668[Abstract/Free Full Text]
  4. Dunnick NR, Korobkin M. Imaging of adrenal incidentalomas: current status. AJR 2002;179 :559-568[Free Full Text]


INSTRUCTIONS
Top
Abstract
INTRODUCTION
EDUCATIONAL OBJECTIVES
REQUIRED READING
INSTRUCTIONS
References
 

  1. Complete the required reading.
  2. Visit www.arrs.org and select the Journals, then Integrative Imaging links from the left side of the home page.
  3. Using your member login, order the online SAM as directed.
  4. Follow the online instructions for entering your responses to the self-assessment questions and complete the evaluation survey.


QUESTION 1

Which one of the following radiologic features, if present, may be useful in differentiating pheochromocytoma from adrenal adenoma on abdominal CT?

  1. Macroscopic fat.
  2. High (> 30 H) attenuation.
  3. Greater than 80% washout.
  4. Calcification.
  5. Arterial phase enhancement.

QUESTION 2

Which examination is contraindicated in the workup of suspected pheochromocytoma?

  1. Abdominal MRI.
  2. Urine vanillylmandelic acid (VMA) measurement.
  3. CT-guided core biopsy.
  4. PET scanning.
  5. Nuclear imaging with iodine-131 metaiodobenzylguanidine (MIBG).

QUESTION 3

Which condition is associated with pheochromocytoma?

  1. Carcinoid syndrome.
  2. Multiple endocrine neoplasias (MEN-2A and MEN-2B).
  3. Osler-Weber-Rendu syndrome.
  4. Congenital adrenal hyperplasia.
  5. Autosomal dominant polycystic kidney disease.

 

Solution to Question 1
Attenuation serves as the principal manner for differentiating benign adenoma and pheochromocytoma [1]. Pheochromocytomas may contain intracellular fat, but only rarely do they contain sufficient lipid to decrease attenuation to less than 10 H. Option B is the best response. Options A, C, and D are incorrect. Although pheochromocytoma can undergo intracellular lipid degeneration, the presence of macroscopic fat (≤ 30 H) is virtually diagnostic of benign myelolipoma. Increased washout is more strongly associated with benign adenoma. With IV contrast injection, both pheochromocytoma and adrenal adenoma show enhancement during the arterial phase. However, pheochromocytoma typically shows washout of less than 40% with a 15-minute delay. Calcification is a rare feature of pheochromocytoma; its presence neither confirms nor excludes the diagnosis.


QUESTION 4

In the evaluation of an incidentally discovered adrenal mass, which feature on MRI, if present, would virtually eliminate pheochromocytoma as a diagnostic possibility?

  1. Cystic morphology.
  2. High signal intensity on T2-weighted imaging.
  3. Heterogeneous enhancement after administration of gadolinium.
  4. Absence of lipid on chemical shift MRI.
  5. Size less than 5 cm.

QUESTION 5

Which of the following incidentally discovered adrenal lesions has specific features on CT that may allow radiologic diagnosis?

  1. Granulomatous disease.
  2. Hemangioma.
  3. Ganglioneuroma.
  4. Pheochromocytoma.
  5. Myelolipoma.

QUESTION 6

Which of the following characteristics of an adrenal adenoma may reliably distinguish it from an adrenal metastasis?

  1. Characteristic pattern of calcification.
  2. Hyperattenuation on unenhanced CT.
  3. Presence of lipid on chemical shift MRI.
  4. Persistence of enhancement on delayed enhanced CT.
  5. Low signal intensity on T2-weighted MRI.

 

Solution to Question 2
CT-guided core biopsy should NOT be contemplated or performed when pheochromocytoma is suspected. Manipulation of these catecholamine-secreting tumors can precipitate life-threatening hypertension or cardiac arrhythmias [2]. An accurate diagnosis must be made through less invasive means. Option C is the best response. If an adrenal mass is not visualized on CT, then MRI is a reasonable choice for a subsequent study. Pheochromocytoma typically has low intensity on T1-weighted images and high intensity on T2-weighted images. The 24-hour measurement of urine VMA and metanephrine levels is an appropriate screening test for pheochromocytoma. These tests should be considered in younger patients with paroxysmal hypertension. For patients in whom CT and MRI do not reveal an adrenal mass despite a high level of clinical and laboratory suspicion, PET is a reasonable alternative. Other useful nuclear imaging studies include MIBG and octreotide.

Solution to Question 3
Approximately 90% of pheochromocytomas are sporadic, and about 10% are related to hereditary conditions. Multiple endocrine neoplasias (MEN-2A and MEN-2B), von Hippel-Lindau disease, von Recklinghausen's disease or neurofibromatosis, and Carney's triad are all associated with the occurrence of pheochromocytomas [3]. Carney's triad consists of gastric stromal tumor, extraadrenal paraganglioma (usually functional), and pulmonary chondroma. Carcinoid syndrome is associated with carcinoid tumors that secrete excessive amounts of serotonin; manifestations may include low blood pressure. Carcinoid syndrome does not occur with pheochromocytoma. Option A is not the best response. Osler-Weber-Rendu syndrome, or hereditary hemorrhagic telangiectasia, is a condition in which patients may have some combination of spontaneous recurrent nosebleeds, mucocutaneous telangiectasia, visceral involvement, and an affected first-degree relative [4], but this condition is not in any way associated with pheochromocytoma. Option B is the best response. Therefore, Option C is not the best response. Congenital adrenal hyperplasia is a group of heritable disorders characterized by cortisol and aldosterone deficiencies and overproduction of androgens; this condition is not associated with pheochromocytoma. Option D is not the best response. Autosomal polycystic kidney disease does not have adrenal manifestations. Option E is not the best response.

Solution to Question 4
On MRI, pheochromocytomas are solid masses that generally have high signal intensity on T2-weighted images and heterogeneous enhancement after the IV administration of gadolinium. Unlike adrenal adenomas, there should be an absence of lipid on chemical shift MRI [5]. Thus, Options B, C, and D are correct features of pheochromocytoma on MRI, and are not the best responses. Size is not a useful criterion for distinguishing pheochromocytoma from other adrenal masses. Option E is not the best response. Pheochromocytomas do not have a cystic morphology. Option A is the best response.

Solution to Question 5
Myelolipomas are benign tumors composed of bone marrow elements and generally present incidentally on imaging of the abdomen performed for other reasons [1]. Myelolipomas generally contain a large amount of mature fat, allowing a confident imaging diagnosis on CT. Myelolipomas occasionally do not contain fat, in which case an imaging diagnosis cannot be made. Option E is the best response. CT findings of granulomatous diseases involving the adrenals, such as tuberculosis and histoplasmosis, are nonspecific and include soft-tissue masses, cystic changes, calcifications, or some combination of these findings. Adrenal hemangiomas are rare benign tumors that are seen on CT as large, well-defined masses of soft-tissue density that exhibit inhomogeneous enhancement after contrast injection. Ganglioneuromas are benign neural tumors that present as solid masses and show variable enhancement with contrast material. Approximately 10% of pheochromocytomas are clinically silent; most of these are discovered as incidental adrenal masses with nonspecific CT characteristics. Options A, B, C, and D are incorrect responses.

Solution to Question 6
Most adrenal adenomas are lipid-rich and can be correctly diagnosed on chemical shift MRI [5]. Option C is the best response. The presence of lipid may also result in adenomas being hypoattenuating relative to normal adrenal tissue on unenhanced CT [6]. Metastases do not contain fat. Calcification in adrenal adenomas is rare, and the presence or absence of calcification does not reliably distinguish these from metastases. Most lipid-poor adenomas can be accurately characterized on delayed enhanced CT, in which there is more rapid washout of contrast material from adenomas than from metastases. Signal intensity of adenomas on T2-weighted MRI tends to be low but overlaps that of metastases; therefore, signal intensity alone is not a reliable method of differentiation. Options A, B, D, and E are incorrect responses.


References
Top
Abstract
INTRODUCTION
EDUCATIONAL OBJECTIVES
REQUIRED READING
INSTRUCTIONS
References
 

  1. Dunnick NR, Korobkin M. Imaging of adrenal incidentalomas: current status. AJR 2002;179 : 559-568[Free Full Text]
  2. Casola G, Nicolet V, van Sonnenberg E, et al. Unsuspected pheochromocytoma: risk of blood-pressure alterations during percutaneous adrenal biopsy. Radiology 1986;159 : 733-735[Abstract/Free Full Text]
  3. Neumann HP, Berger DP, Sigmund G, et al. Pheochromocytomas, multiple endocrine neoplasia type 2, and von Hippel-Lindau disease. N Engl J Med 1993;329 : 1531-1538[Abstract/Free Full Text]
  4. Begbie ME, Wallace GMF, Shovlin CL. Hereditary haemorrhagic telangiectasia (Osler-Weber-Rendu syndrome): a view from the 21st century. Postgrad Med J 2003;79 : 18-24[Abstract/Free Full Text]
  5. Elsayes KM, Narra VR, Leyendecker JR, Francis IR, Lewis JS Jr, Brown JJ. MRI of adrenal and extraadrenal pheochromocytoma. AJR 2005; 184:860 -867[Free Full Text]
  6. Blake MA, Krishnamoorthy SK, Boland GW, et al. Low-density pheochromocytoma on CT: a mimicker of adrenal adenoma. AJR 2003; 181:1663 -1668[Abstract/Free Full Text]

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This Article
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