Division of Pediatric Surgery, Michael E. DeBakey Department of Surgery, Baylor College of Medicine, Houston, TX, USA.
J Pediatr Surg. 2013 Jun;48(6):1422-5. doi: 10.1016/j.jpedsurg.2013.04.001.
Management of pediatric patients with pheochromocytomas associated with genetic syndromes, such as Von Hippel-Lindau (VHL) disease, is complex owing to the need for continuous surveillance, high rates of recurrence, multiple operations during childhood, and the possibility of lifelong exogenous steroid replacement. We report the successful treatment of two siblings with VHL who underwent a total of six cortical-sparing procedures without requiring steroid therapy.
管理与遗传综合征相关的儿童嗜铬细胞瘤患者（如 von Hippel-Lindau [VHL] 病）非常复杂，这是由于需要持续监测、高复发率、儿童期多次手术以及终身需要外源性激素替代的可能性所致。我们报告了两例 VHL 同胞患者的成功治疗，他们总共进行了 6 次皮质保留手术，而无需接受类固醇治疗。
