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      Drugs & Diseases > Endocrinology

      Pheochromocytoma Treatment & Management

      Updated: Aug 20, 2021
      • Author: Michael A Blake, MBBCh, MRCPI, FRCR; Chief Editor: George T Griffing, MD  more...
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      Treatment

      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) [34, 35] :

      • 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. [57]  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. [58]

      A retrospective study by Butz et al found that among patients with pheochromocytoma/paraganglioma associated with neurofibromatosis type 1 (NF1), multiple endocrine neoplasia type 2A (MEN 2A), or von Hippel-Lindau (VHL) disease, the most volatile intraoperative course hemodynamically, as well as more severe postoperative complications, occurred in patients with NF1. The investigators attributed this to the presence of large tumors secreting great amounts of catecholamine in these patients, along with the high proportion of the NF1 patients who were treated with open resection. Postoperative outcomes between the three groups did not differ significantly for patients who were treated laparoscopically. [59]

      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. [60]  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.

      A retrospective study by Araujo-Castro et al reported that in patients with pheochromocytomas, a greater risk of intraoperative complications can be linked to higher urine metanephrine and normetanephrine levels, greater tumor size, a presurgical blood pressure of more than 130/80 mmHg, pretreatment with doxazosin, and an absence of perioperative hydrocortisone therapy. For example, 51.1% of patients in the study who were pretreated with doxazosin needed intraoperative hypotensive treatment, compared with 26.5% of those who underwent pretreatment with other antihypertensive agents. [61]

      No distinction is found in hypertensive episodes during surgery for pheochromocytoma associated with 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. [62]

      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.

      A literature review by Holscher et al indicated that following curative adrenalectomy, the pooled recurrence rate for sporadic pheochromocytomas is 3%. The weighted mean time to recurrence in the study was 49.4 months, with the weighted mean follow-up period being 77.3 months. [63]

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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. [6, 64, 65]

      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. [66]

      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%). [6]  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 postpartum. [29]

      A study by Donatini et al suggested that in pregnant women with bilateral pheochromocytomas, unilateral adrenalectomy for the larger tumor during pregnancy may be a useful strategy to avoid adrenal insufficiency, with contralateral adrenalectomy performed a few months after delivery. [67]

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