Title |
Co-Occurrence of Pheochromocytoma-Paraganglioma and Cyanotic Congenital Heart Disease: A Case Report and Literature Review
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Published in |
Frontiers in endocrinology, April 2018
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DOI | 10.3389/fendo.2018.00165 |
Pubmed ID | |
Authors |
Bingbin Zhao, Yi Zhou, Yi Zhao, Yumo Zhao, Xingcheng Wu, Yalan Bi, Yufeng Luo, Zhigang Ji, Shi Rong |
Abstract |
Pheochromocytoma and paraganglioma (PHEO-PGL) and cyanotic congenital heart disease (CCHD) are both rare diseases. We reported a 30-year-old patient with a right adrenal gland nodule and a retroperitoneal mass and history of functional single atrium and ventricle. 123I-metaiodobenzylguanidine scintigraphy showed intense uptake in both lesions. Laboratory investigation demonstrated elevated urinary norepinephrine. Preoperative α-blockade was initiated. A successful open resection of right adrenal and retroperitoneal masses was performed. Pathological examination confirmed PHEO-PGL. Postoperative urinary norepinephrine returned to normal level. A systematic case review in English publications in PubMed and EMBASE suggested a hypothesis that there may exist a possible link between PHEO-PGL and hypoxia from CCHD, which was also indicated in our case. Due to higher risk for PHEO-PGL, a lower threshold of suspicion should be considered in CCHD patients. Therefore, active screening and early treatment of PHEO-PGL are recommended in CCHD patients and clinicians should keep on a long-term follow-up to monitor PHEO-PGL recurrence if hypoxia is not corrected. |
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Mendeley readers
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Student > Master | 2 | 11% |
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Materials Science | 1 | 5% |
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Unknown | 4 | 21% |