Partial Adrenalectomy Minimizes the Need for Long-Term Hormone Replacement in Pediatric Patients with Pheochromocytoma and von Hippel-Lindau Syndrome

摘要

PurposeChildren with von Hippel-Lindau syndrome are at an increased risk for developing bilateral pheochromocytomas. In an effort to illustrate the advantage of partial adrenalectomy (PA) over total adrenalectomy in children with VHL, we report the largest single series on PA for pediatric VHL patients, demonstrating a balance between tumor removal and preservation of adrenocortical function.