Approach to the Preoperative Pharmacological Management of Catecholamine-Secreting Tumors in Children: A Case Series and Review

Catecholamine-secreting tumors (CSTs) are an uncommon tumor in pediatrics that can result in hypertensive crisis secondary to catecholamine release from the tumor cells. Pheochromocytoma is the tumor type most associated with excessive catecholamine release, but paragangliomas and neuroblastoma tumors can also generate and release excessive amounts of catecholamines. Surgical resection is typically the treatment of choice for these tumor types; however, manipulation during surgical resection can lead to further catecholamine release. If the patient's adrenergic receptors are not fully inhibited before undergoing surgery, the patient is at risk for experiencing additional hypertensive crises and arrhythmias due to the catecholamine release secondary to the surgery. Although studies have identified sequential α- and β-adrenergic blockade as a prerequisite for surgery to minimize the effects of further catecholamine release during resection, there is little guidance regarding medication timing, dosing, and testing to ensure the pediatric patient has adequately suppressed adrenergic receptors before surgery. Further, with recent shortages of the first-line medication treatment options for this indication, it is vital to establish a treatment plan with alternative treatments for use during drug shortages. This case series describes the successful use of enteral phenoxybenzamine and parenteral phentolamine for α-adrenergic blockade, coupled with enteral atenolol and parenteral esmolol or labetalol for β-adrenergic blockade, in 9 pediatric patients with CST before surgical resection of the tumors. Eight patients underwent a phenylephrine challenge, and all demonstrated appropriate α-blockade preceding tumor removal.