Acute Adrenal Conditions: Pheochromocytoma Emergencies

Pheochromocytoma and paragangliomas (PPGL) are rare chromaffin cells tumor characterized by the production of catecholamines and that can be fatal if left undiagnosed. These neuroendocrine tumors are often misdiagnosed and consequently not adequately treated. Characteristically, patients present with hypertension (sustained or, most often, paroxysmal), usually associated with the classic triad of headache, palpitations, and sweating. Nevertheless, these lesions may present in several possible situations that could evolve suddenly to true emergency conditions, mostly related to the cardiovascular system, such as hypertensive crises or arrhythmias, or even multisystemic crises. In this situation, the clinical suspicion is an absolute cornerstone of the management and an eventual delay in diagnosis is adversely proportional to the overall outcome. Patients who present with PPGL crisis should be admitted to the hospital for aggressive medical management of symptoms before surgical treatment. In these cases, immediate surgical intervention without stabilization of vital parameters is associated with high morbidity and mortality. Besides, although many of these patients can be stabilized by means of alpha-adrenoreceptor blockers, a multidisciplinary approach is mandatory. All in all, adrenalectomy can be performed within 1–2 weeks in patients who generally recover with medical and intensive care support.