[Diagnostics and treatment of pheochromocytomas and paragangliomas]

Pheochromocytomas and paragangliomas (PPGL) are rare neuroendocrine tumors arising from chromaffin cells and are capable of producing catecholamines. The diagnostics rely on measurements of free plasma and urinary fractionated metanephrines, complemented by computed tomography (CT) or magnetic resonance imaging (MRI) for anatomical imaging. Up to 35% of PPGLs are hereditary and the underlying genetic background significantly influences both tumor biology and surgical strategy. Surgery remains the cornerstone of treatment. For adrenal tumors, laparoscopic or retroperitoneoscopic adrenalectomy represents the standard of care, while minimally invasive approaches are also feasible for many paragangliomas. Preoperative blood pressure optimization is essential, although the routine use of an alpha-blockade is increasingly questioned. Organ-preserving tumor extirpation should be considered whenever oncologically safe, particularly in patients with hereditary syndromes, bilateral tumors and younger age, in order to avoid postoperative adrenal insufficiency.