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Surgery
Source: Pheochromocytoma and Paraganglioma Treatment (PDQ®)–Health Professional Version, National Cancer Institute.
Source updated: November 1, 2024 · Captured 2026-09-09.
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Context: Treatment of Localized Pheochromocytoma / Treatment Options for Inherited Pheochromocytoma
The surgical management of pheochromocytoma in patients with the hereditary syndromes multiple endocrine neoplasia type 2 (MEN2) or 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]
Recommendations generally favor preservation of adrenal cortical tissue in patients with MEN2 or VHL 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.[3]
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Evidence (surgery):
A single-institution study included 56 patients with adrenal pheochromocytomas.[5]
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A single-institution study included 56 patients with adrenal pheochromocytomas.[5]
Of the 30 patients who underwent one or more cortical-sparing adrenalectomies, 17 (57%) avoided the need for routine steroid replacement.
A single-institution study included 56 patients with adrenal pheochromocytomas.[5]
The clinical recurrence rate was low (3 of 30 patients) and none of the patients developed metastatic disease.[5][Level of evidence C2]
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A similar approach may be reasonable in other hereditary pheochromocytoma-paraganglioma syndromes that are characterized by benign disease, but there are insufficient data upon which to base unequivocal recommendations. For more information, see Genetics of Endocrine and Neuroendocrine Neoplasias.
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Publication references
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Preserved source evidence · Not medical advice