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Pheochromocytoma Treatment & Management

  • Author: Michael A Blake, MBBCh; Chief Editor: George T Griffing, MD  more...
 
Updated: Jun 30, 2016
 

Approach Considerations

The Endocrine Society, the American Association for Clinical Chemistry, and the European Society of Endocrinology have released clinical practice guidelines for the diagnosis and management of pheochromocytoma and paraganglioma (jointly referred to as PPGL).[26, 27, 27] :

  • Preoperative blockade of hormonally functional PPGL to prevent cardiovascular complications is recommended, along with preoperative medical treatment to normalize blood pressure and heart rate and a high-sodium diet with fluid intake to prevent severe hypotension after removal of the tumor.
  • Blood pressure, heart rate, and glucose levels should be monitored immediately after surgery.
  • Minimally invasive (eg, laparoscopic) adrenalectomy should be performed for most adrenal pheochromocytomas, with open resection reserved for very large or invasive pheochromocytomas; open resection is suggested for paragangliomas, but laparoscopic resection is an option for smaller tumors; partial adrenalectomy is also an option for certain patients.

Surgical resection of the tumor is the treatment of choice for pheochromocytoma and usually results in cure of the hypertension. Careful preoperative management is required to control blood pressure, correct fluid volume, and prevent intraoperative hypertensive crises.[47]  A study by Kwon et al indicated that independent risk factors for a hypertensive attack during adrenalectomy for pheochromocytoma include a large tumor size and preoperative elevation of the urinary epinephrine level.[48]

Although there is no consensus regarding the preferred drugs for preoperative blood pressure control, alpha blockers, beta blockers, calcium channel blockers, and angiotensin receptor blockers have all been used.[49] Start alpha blockade with phenoxybenzamine 10-14 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 (usually, 2 days). 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 for pheochromocytoma associated with multiple endocrine neoplasia type 2 (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.[50]

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

For surgical follow-up, obtain plasma metanephrine levels yearly for 10 years. Ensure that blood pressure is under control. In patients with an underlying genetic mutation, lifelong follow-up is mandatory.

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Laparoscopic Adrenalectomy

Surgical mortality rates are less than 2-3% when the operation is performed by a surgeon and an anesthesiologist who are experienced.

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; in current practice, however, laparoscopic adrenalectomy is the preferred procedure for lesions smaller than 8 cm. If the pheochromocytoma is intra-adrenal, the standard approach is to remove the entire adrenal gland. In the case of a malignant pheochromocytoma, resect as much of the tumor as possible.[3, 51, 52]

A study by Scholten et al found that unilateral subtotal adrenalectomy is a feasible strategy in patients with MEN2 who have pheochromocytoma. It has comparable recurrence rates and less complications of steroid replacement compared with unilateral total adrenalectomy.[53]

In a study by Paraby et al, severe hypertension (systolic blood pressure >200 mm Hg) was associated with 5 of 35 pheochromocytoma resections (14.3%), compared with 2 of 106 nonpheochromocytoma adrenal tumor resections (1.9%).[3] However, no patient in either group had transient or persistent systolic blood pressure of greater than 220 mm Hg. There were no significant differences in recovery room hemodynamic parameters, frequency of persistent hypotension, or occurrence of heart rates greater than 120/min between the 2 groups.

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Pheochromocytoma in Pregnancy

If pheochromocytoma is found during pregnancy, initiate alpha-adrenergic blockade (with phenoxybenzamine) as soon as the diagnosis is confirmed. Remove the tumor by laparoscopic adrenalectomy as soon as possible during the first 2 trimesters, after proper preparation. Pregnancy need not be terminated. Spontaneous abortion is very likely, however.

During the third trimester, the patient should be managed medically until fetal lung maturity is confirmed. Cesarean delivery is preferred, as mortality may be higher with vaginal delivery. The tumor may be removed during the same session as the cesarean section, or it can be removed post partum.[24]

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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 is a member of the following medical societies: American College of Radiology, American Roentgen Ray Society, Radiological Society of North America, Royal College of Surgeons in Ireland, Royal College of Physicians of Ireland

Disclosure: Received royalty from Springer for 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, Endocrine Society

Disclosure: Nothing to disclose.

Chief Editor

George T Griffing, MD Professor Emeritus 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, International Society for Clinical Densitometry, Southern Society for Clinical Investigation, American College of Medical Practice Executives, American Association for Physician Leadership, American College of Physicians, American Diabetes Association, American Federation for Medical Research, American Heart Association, Central Society for Clinical and Translational Research, Endocrine Society

Disclosure: Nothing to disclose.

Acknowledgements

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.

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

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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.
Adrenalectomy specimen containing pheochromocytoma. Non-neoplastic adrenal cortex (yellow) surrounds a small tan-red tumor in the medullary region, representing a pheochromocytoma.
H and E, high power, showing classic "balls of cells" feature of a pheochromocytoma. Endocrine tumors such as a pheochromocytoma typically show some degree of nuclear pleomorphism ("endocrine atypia") which does not indicate malignancy.
 
 
 
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