Pheochromocytoma Treatment & Management

  • Author: Michael A Blake, MBBCh, MRCPI, FRCR; Chief Editor: George T Griffing, MD   more...
 
Updated: Dec 12, 2011
 

Approach Considerations

Surgical resection of the tumor is the treatment of choice and usually results in cure of the hypertension. Careful treatment with alpha and beta blockers is required preoperatively to control blood pressure and prevent intraoperative hypertensive crises.[21]

Start alpha blockade with phenoxybenzamine 7-10 days preoperatively to allow for expansion of blood volume. The patient should undergo volume expansion with isotonic sodium chloride solution. Encourage liberal salt intake.

Initiate a beta blocker only after adequate alpha blockade. If beta blockade is started prematurely, unopposed alpha stimulation could precipitate a hypertensive crisis. Administer the last doses of oral alpha and beta blockers on the morning of surgery. No distinction is found in hypertensive episodes during surgery between MEN 2 and non-MEN–associated pheochromocytoma. Therefore, pretreatment using alpha and beta adrenergic blockers remains a standard of care in both groups of patients.[22]

Postoperative testing

Test plasma free metanephrines 2 weeks postoperatively. If results are within the reference range, resection is deemed complete and patient survival approaches age-matched controls. In addition, assure resolution of the hypertension and any associated complications.

Long-term postoperative monitoring

Obtain plasma metanephrine levels yearly for 5 years. Assure that blood pressure is under control.

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Laparoscopic Adrenalectomy

An experienced anesthesiologist and an experienced surgeon are crucial to the success of the operation. Surgical mortality rates are less than 2-3% with an experienced anesthesiologist and surgeon.

Use an arterial line, cardiac monitor, and Swan-Ganz catheter. Administer stress-dose steroids if bilateral resection is planned.

An anterior midline abdominal approach was used in the past; however, in current practice, laparoscopic adrenalectomy is the preferred procedure for lesions smaller than 8 cm. If the pheochromocytoma is intra-adrenal, remove the entire adrenal gland. In the case of a malignant pheochromocytoma, resect as much of the tumor as possible.[3, 23, 24]

A study by Scholten et al found that unilateral subtotal adrenalectomy is a feasible strategy in patients with multiple endocrine neoplasia 2 (MEN2) who have pheochromocytoma. It has comparable recurrence rates and less complications of steroid replacement compared with unilateral total adrenalectomy.[25]

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Pheochromocytoma in Pregnancy

If pheochromocytoma is found during pregnancy, administer alpha-adrenergic blockade (phenoxybenzamine) as soon as the diagnosis is confirmed. Surgically remove the tumor as soon as possible during the first 2 trimesters after proper preparation. Pregnancy need not be terminated. Spontaneous abortion is very likely.

During the third trimester, as soon as fetal lung maturity is confirmed, perform surgical removal of the tumor and follow with cesarean delivery.

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Contributor Information and Disclosures
Author

Michael A Blake, MBBCh, MRCPI, FRCR  Assistant Professor, Department of Radiology, Harvard Medical School; Staff Radiologist, Division of Abdominal Imaging, Massachusetts General Hospital

Michael A Blake, MBBCh, MRCPI, FRCR is a member of the following medical societies: American College of Radiology, American Roentgen Ray Society, Radiological Society of North America, Royal College of Physicians of Ireland, and Royal College of Surgeons in Ireland

Disclosure: Springer Royalty book editor

Coauthor(s)

Ann T Sweeney, MD  Associate Professor, Department of Medicine, Division of Endocrinology, Tufts University School of Medicine

Ann T Sweeney, MD is a member of the following medical societies: American Association of Clinical Endocrinologists and Endocrine Society

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Romesh Khardori, MD, PhD, FACP  Professor of Endocrinology, Director of Training Program, Division of Endocrinology, Diabetes and Metabolism, Strelitz Diabetes and Endocrine Disorders Institute, Department of Internal Medicine, Eastern Virginia Medical School

Romesh Khardori, MD, PhD, FACP is a member of the following medical societies: American Association of Clinical Endocrinologists, American College of Physicians, American Diabetes Association, and Endocrine Society

Disclosure: Nothing to disclose.

Chief Editor

George T Griffing, MD  Professor of Medicine, St Louis University School of Medicine

George T Griffing, MD is a member of the following medical societies: American Association for the Advancement of Science, American College of Medical Practice Executives, American College of Physician Executives, American College of Physicians, American Diabetes Association, American Federation for Medical Research, American Heart Association, Central Society for Clinical Research, Endocrine Society, International Society for Clinical Densitometry, and Southern Society for Clinical Investigation

Disclosure: Nothing to disclose.

Additional Contributors

The authors and editors of Medscape Reference gratefully acknowledge the contributions of previous author James C Melby, MD, to the development and writing of the source article.

References

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  16. Baid SK, Lai EW, Wesley RA, Ling A, Timmers HJ, Adams KT, et al. Brief communication: radiographic contrast infusion and catecholamine release in patients with pheochromocytoma. Ann Intern Med. Jan 6 2009;150(1):27-32. [Medline].

  17. Bessell-Browne R, O'Malley ME. CT of pheochromocytoma and paraganglioma: risk of adverse events with i.v. administration of nonionic contrast material. AJR Am J Roentgenol. Apr 2007;188(4):970-4. [Medline]. [Full Text].

  18. Blake MA, Kalra MK, Maher MM, Sahani DV, Sweeney AT, Mueller PR, et al. Pheochromocytoma: an imaging chameleon. Radiographics. Oct 2004;24 Suppl 1:S87-99. [Medline].

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  20. Kim S, Salibi N, Hardie AD, Xu J, Lim RP, Lee VS, et al. Characterization of adrenal pheochromocytoma using respiratory-triggered proton MR spectroscopy: initial experience. AJR Am J Roentgenol. Feb 2009;192(2):450-4. [Medline].

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  22. Scholten A, Vriens MR, Cromheecke GJ, Borel Rinkes IH, Valk GD. Hemodynamic instability during resection of pheochromocytoma in MEN versus non-MEN patients. Eur J Endocrinol. Jul 2011;165(1):91-6. [Medline].

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Axial, T2-weighted magnetic resonance imaging (MRI) scan showing large left suprarenal mass of high signal intensity on a T2-weighted image. The mass is a pheochromocytoma.
Abdominal computed tomography (CT) scan demonstrating left suprarenal mass of soft-tissue attenuation representing a paraganglioma.
 
 
 
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