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Case Reports
. 2018 May-Aug;11(2):197-200.
doi: 10.4103/apc.APC_172_17.

Retroperitoneal paraganglioma in a patient with Fontan: The hypoxia connection

Shriprasad R Deshpande  1 , Priyanka Patel  2 , Neill Videlefsky  3 , Dellys M Soler Rodriguez  4 , Rene Romero  4 , Matthew S Clifton  5
Affiliations
Case Reports

Retroperitoneal paraganglioma in a patient with Fontan: The hypoxia connection

Shriprasad R Deshpande et al. Ann Pediatr Cardiol. 2018 May-Aug.

Abstract

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.

Keywords: Congenital heart disease; Fontan; heart transplantation; hypoxia; outcome; paraganglioma.

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Conflict of interest statement

There are no conflicts of interest.

Figures

Figure 1
Figure 1
Magnetic resonance imaging of the abdomen showing coronal (a) and sagittal (b) view of the heterogeneous mass (arrows) appearing to arise from the pancreas, close to the right lobe of the liver, and immediately posterior to the duodenum
Figure 2
Figure 2
Histopathology of the paraganglioma. (a) H and E stain showing well-defined nests of cuboidal cells with abundant cytoplasm separated by highly vascularized fibrous septa. (b) Immunostaining shows strong positivity to synaptophysin immunostain in the tumor cells
Figure 3
Figure 3
Liver biopsy histopathology demonstrating (a) show hepatic lobules and portal areas with fibrosis with H and E staining while (b) staining with Masson's trichrome stain highlights the extent of bridging fibrosis consistent with Fontan hepatopathy
See this image and copyright information in PMC

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