Department of Urology, University of Kentucky, Lexington, Kentucky.
Division of Urology, Department of Surgery, University of Colorado and Children's Hospital Colorado, Aurora, Colorado, USA.
Curr Opin Urol. 2019 Sep;29(5):493-499. doi: 10.1097/MOU.0000000000000650.
To provide an overview of relevant data available and updated recommendations for management of pediatric patients with pheochromocytoma (PCC).
Much of the available data surrounding pediatric PCC is in the form of case reports and case series. With the accumulation of data over time, pediatric PCC does in fact differ significantly from not only what is known in the adult population, but also from classic teaching. Pediatric patients are much more likely to have a hereditary predisposition as well as aggressive and malignant disease. Much of the recent literature focuses on defining these genetic syndromes in order to provide recommendations for screening and genetic counseling. Other recent advances center around developing treatments for metastatic disease. Timely diagnosis with plasma metanephrines and cross-sectional imaging, and appropriate preoperative medical optimization followed by surgical resection remain the center of treatment.
Although rare and adult principles are applied to pediatric PCC, genetic testing plays a pivotal role in management of children, adolescents and young adults with PCC.
提供有关儿童嗜铬细胞瘤（PCC）管理的现有相关数据和更新建议的概述。
围绕儿科 PCC 的大量可用数据以病例报告和病例系列的形式存在。随着时间的推移数据的积累，儿科 PCC 确实与成人人群中的情况以及经典教学有很大的不同。儿科患者更有可能存在遗传易感性以及侵袭性和恶性疾病。最近的大部分文献都集中在定义这些遗传综合征上，以便为筛查和遗传咨询提供建议。其他最近的进展集中在开发转移性疾病的治疗方法上。及时诊断血浆代谢产物和横断面成像，以及适当的术前药物优化，然后进行手术切除仍然是治疗的核心。
尽管罕见且适用于成人的原则也适用于儿科 PCC，但基因检测在患有 PCC 的儿童、青少年和年轻人的管理中起着关键作用。
