Related Experiment Video
Updated: Sep 19, 2025
A Novel Method: Super-selective Adrenal Venous Sampling
Published on: September 15, 2017
Partial adrenalectomy for pheochromocytoma in the multiple endocrine neoplasia type 2 population: Time to reconsider?
Diederik P D Suurd1, Medard F M Van den Broek2, Charlotte L Viëtor3
1Department of Surgical Oncology and Endocrine Surgery, University Medical Center Utrecht, Utrecht, the Netherlands.
Background:
Adrenalectomy is considered the standard of care for multiple endocrine neoplasia type 2-related pheochromocytomas. Recently, partial adrenalectomy has been suggested as an alternative to prevent adrenal insufficiency in hereditary pheochromocytoma. Nevertheless, this comes with the risk of ipsilateral recurrence. Therefore, we aimed to determine the incidence of ipsilateral recurrence after partial adrenalectomy and assess the proportion of prevented adrenal insufficiency due to partial adrenalectomy in cases of bilateral disease.
Methods:
A retrospective, multicenter cohort study was conducted between 1977 and 2022 via a standardized medical record review. The primary outcome was the incidence of ipsilateral recurrence after partial adrenalectomy compared with total adrenalectomy. The secondary outcomes included disease-free survival and incidence of adrenal insufficiency and crisis after bilateral surgery.
Results:
In 50 patients with multiple endocrine neoplasia type 2, a total of 88 adrenalectomies were performed, including 23 partial adrenalectomies and 65 total adrenalectomies. After a median follow-up after last surgery of 8.3 years, 7 (30.4%) of the partial adrenalectomies and 2 (3.1%) of total adrenalectomies had recurrent disease (P < .001, χ2 test). The median time to recurrence was 4 and 21 years after partial adrenalectomy and total adrenalectomy, respectively. After bilateral surgery with at least partial adrenalectomy on one side, 50.0% of patients did not require corticosteroids.
Conclusion:
These data show that there is a considerable risk of ipsilateral recurrent disease in the short term after partial adrenalectomy for multiple endocrine neoplasia type 2-related pheochromocytoma with a 50% chance of adrenal insufficiency in cases of bilateral surgery. Therefore, we doubt whether partial adrenalectomy should be preferred for multiple endocrine neoplasia type 2-associated pheochromocytoma. We propose total adrenalectomy as the standard technique and partial adrenalectomy only for selected cases.
Related Concept Videos
01:27Adrenal Gland Disorders
Adrenal insufficiency, characterized by insufficient cortisol and aldosterone production, leads to conditions like Addison's disease. This disorder, affecting the adrenal cortex, exhibits symptoms such as skin bronzing, dehydration, low blood pressure, fatigue, and weight loss. Congenital adrenal hyperplasia, a genetic ailment causing...
01:31Adrenergic Receptors: ɑ Subtype
Adrenaline ≥ Noradrenaline >> Isoprenaline
α-adrenoceptors are further divided into α1 and α2-adrenoceptors.
α1-Adrenoceptors: These receptors are located postsynaptically on the effector organs and cause constriction of smooth muscle mediated by activation of phospholipase...
01:22Adrenergic Antagonists: Pharmacological Actions of ɑ-Receptor Blockers
α1-blockers: These drugs inhibit α1-adrenoceptors on smooth muscle cells, resulting in vasodilation. This vasodilation lowers blood pressure, making α1-blockers valuable in treating hypertension. Additionally,...
01:30Adrenergic Agonists: Therapeutic Uses
Emergency and Intensive Care Unit (ICU) applications: Pressor agents increase blood pressure, heart rate, and contractility in shock and organ failure situations. Dopamine can induce vasodilation and stimulate adrenoceptors. Endogenous catecholamines are effective in treating cardiogenic shock. α2-agonists like clonidine can reverse anesthesia-induced hypertension.
Allergies and...
01:27Adrenergic Receptors (Adrenoceptors): Classification
α-Adrenoceptors
α-Adrenoceptors are classified into two main subtypes: α1 and α2. The α1 adrenoceptors,...
01:27Adrenergic Antagonists: Pharmacological Actions of β-Receptor Blockers