Pheochromocytoma and Paraganglioma Treatment (PDQ®): Treatment - Health Professional Information [NCI] - Localized Pheochromocytoma Treatment
The standard treatment option for patients with localized pheochromocytoma is surgery.
Surgical resection, i.e., adrenalectomy, is the definitive treatment for patients with localized pheochromocytoma. A minimally invasive adrenalectomy is the generally preferred approach if the following conditions can be met:
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Preoperative imaging reveals an adrenal pheochromocytoma that is approximately 6 cm or smaller in diameter.
No radiographic evidence of invasion into adjacent structures or evidence of regional or metastatic disease (i.e., presumably a benign tumor).
Normal contralateral adrenal gland.
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 standard treatment option for patients with inherited pheochromocytoma is surgery.
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. (Refer to the PDQ summary on the Genetics of Medullary Thyroid Cancer for more information on the treatment of inherited pheochromocytoma.)