Pheochromocytomas and paragangliomas (PPGLs) are rare neuroendocrine tumors that secrete excess catecholamines leading to secondary hypertension and cardiovascular morbidity. Once biochemical testing with either 24-hour urinary fractioned metanephrines or plasma free metanephrines confirms the diagnosis, patients are optimized with adequate hydration to maintain their intravascular volume and the appropriate antihypertensive medications are initiated for optimal blood pressure control. Genetic testing and imaging is performed to determine the extent of adrenalectomy and the optimal surgical approach. Surgical approaches include transabdominal or retroperitoneal minimally invasive approaches, and transabdominal open approaches. Factors that influence the surgical approach include germline genetic test results, the size of the tumor, body mass index, surgeon experience, and the likelihood of malignancy. The extent of adrenalectomy is based on germline genetic findings. Patients with syndromes such as von Hippel Lindau (VHL) or multiple endocrine neoplasia 2 (MEN 2) benefit from cortical-sparing adrenalectomy to avoid chronic steroid replacement and the risk of Addisonian crisis. Postoperative management includes hemodynamic monitoring and assessment for signs of hypoglycemia. Outcomes after surgery show improved blood pressure control in most patients and normalization of blood pressure in about a third of patients. Long-term follow-up is required for all patients to assess for recurrence.
Keywords: Pheochromocytoma; approach; management; outcomes; surgery.
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