Note: Some citations in the text of this section are followed by a level of evidence. The PDQ editorial boards use a formal ranking system to help the reader judge the strength of evidence linked to the reported results of a therapeutic strategy. (Refer to the PDQ summary on Levels of Evidence for more information.)
Purpose of This Summary
This PDQ cancer information summary for health professionals provides comprehensive, peer-reviewed, evidence-based information about the pathophysiology and treatment of gastrointestinal complications, including constipation, impaction, bowel obstruction, diarrhea, and radiation enteritis. It is intended as a resource to inform and assist clinicians who care for cancer patients. It does not provide formal guidelines or recommendations for making health care decisions...
Surgical resection, i.e., adrenalectomy, is the definitive treatment for patients with localized pheochromocytoma. If preoperative imaging reveals an adrenal pheochromocytoma that is approximately 6 cm or smaller in diameter, without radiographic evidence of invasion into adjacent structures or evidence of regional or metastatic disease (i.e., presumably a benign tumor), and there is a normal contralateral adrenal gland, a minimally invasive adrenalectomy is the generally preferred approach. Both anterior transabdominal laparoscopic adrenalectomy as well as posterior retroperitoneoscopic adrenalectomy have been demonstrated to be safe for the majority of patients with a modestly sized, radiographically benign pheochromocytoma.[1,2] If preoperative imaging suggests malignancy, or if the patient has an extra-adrenal paraganglioma or multifocal disease, an open approach is generally preferred.
Intraoperative hypertension can be controlled with intravenous infusion of phentolamine, sodium nitroprusside, or a short-acting calcium-channel blocker (e.g., nicardipine). Tumor removal may be followed by a sudden drop in blood pressure that may require rapid volume replacement and intravenous vasoconstrictors (e.g., norepinephrine or phenylephrine). Postoperatively, patients should remain in a monitored environment for 24 hours. Postoperative hypotension is managed primarily by volume expansion, and postoperative hypertension usually responds to diuretics.
The surgical management of pheochromocytoma in patients with the hereditary syndromes multiple endocrine neoplasia type 2 (MEN2) and von Hippel-Lindau (VHL) disease has been controversial. In both of these syndromes, pheochromocytoma is bilateral in at least 50% of patients; however, malignancy is very uncommon. Bilateral total adrenalectomy commits all patients to lifelong steroid dependence, and up to 25% of patients will experience Addisonian crisis (acute adrenal insufficiency).[3,4]
Current recommendations generally favor preservation of adrenal cortical tissue in patients with MEN2 and VHL syndromes when possible. Patients who initially present with unilateral pheochromocytoma should undergo unilateral adrenalectomy, and patients who present with bilateral pheochromocytomas or who develop pheochromocytoma in their remaining adrenal gland should undergo cortical-sparing adrenalectomy, when technically feasible.
In a single-institution study involving 56 patients with pheochromocytoma, 57% of patients (i.e., 17 of 30 patients) who underwent one or more cortical-sparing adrenalectomies avoided the need for routine steroid replacement; the clinical recurrence rate was low (i.e., 3 of 30 patients) and none of the patients developed metastatic disease.[Level of evidence: 3iiDii] A similar approach may be reasonable in other hereditary pheochromocytoma-paraganglioma syndromes that are characterized by benign disease, but there are currently insufficient data upon which to base unequivocal recommendations.