Deshpande Shriprasad R, Patel Priyanka, Videlefsky Neill, Soler Rodriguez Dellys M, Romero Rene, Clifton Matthew S
Department of Pediatrics, Pediatric Cardiology, Heart Failure and Heart Transplantation, Emory University School of Medicine, Atlanta, GA, USA.
Emory University School of Medicine, Atlanta, GA, USA.
Ann Pediatr Cardiol. 2018 May-Aug;11(2):197-200. doi: 10.4103/apc.APC_172_17.
Paragangliomas are rare neuroendocrine tumors where hypoxia-inducible factor plays a critical role in tumorigenesis. It has been suggested that patients with congenital heart disease, in particular, may have cellular environment and relative hypoxia favorable to the development of these neuroendocrine tumors. Here, we present a case of an 11-year-old child with hypoplastic left heart syndrome previously palliated with Fontan procedure, diagnosed with paraganglioma on surveillance imaging. We present the clinical course, intervention, and outcome as well as review the possible contributory mechanisms. As we continue to improve long-term survival for single ventricle patients, awareness of these tumors during surveillance may be warranted as timely intervention may lead to cure.
副神经节瘤是罕见的神经内分泌肿瘤，其中缺氧诱导因子在肿瘤发生中起关键作用。有人提出，特别是先天性心脏病患者，可能具有有利于这些神经内分泌肿瘤发展的细胞环境和相对缺氧状态。在此，我们报告一例11岁患有左心发育不全综合征的儿童病例，该患儿此前接受了Fontan手术姑息治疗，在监测成像时被诊断为副神经节瘤。我们介绍了临床过程、干预措施和结果，并回顾了可能的促成机制。随着我们不断提高单心室患者的长期生存率，在监测期间对这些肿瘤保持警惕可能是必要的，因为及时干预可能会治愈疾病。
