Pheochromocytomas: Surgical Approach

The incidence of pheochromocytoma in von Hippel-Lindau (VHL) disease varies based on specific genotype—approximately 20–30% overall. VHL patients should be screened from a young age, and management should involve multidisciplinary coordination of care. Timing, type of surgery, and surgical approach should be personalized to each patient and the manifestation of his or her disease, as VHL patients are at an increased risk for bilateral and multifocal pheochromocytomas, as well as tumors in the nearby kidney and pancreas. Close coordination between medical, surgical, and anesthesia teams is crucial in the perioperative period. Following resection, patients undergo lifelong surveillance for recurrent pheochromocytoma.