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Salvatore M. Corsello, Rosa Maria Paragliola, Pietro Locantore, Germano Perotti, Francesco Musumeci, Alfredo Pontecorvi, Retrocardiac Catecholamine-Producing Paraganglioma, The Journal of Clinical Endocrinology & Metabolism, Volume 96, Issue 9, 1 September 2011, Pages 2663–2664, https://doi.org/10.1210/jc.2011-0444
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Paragangliomas (PGLs) represent about 20% of catecholamine-producing tumors (1). As reported by Erickson et al. (2), they are mostly found in the head and neck, whereas the retrocardial location represents an unusual finding (3–5).
A 41-yr-old woman came to our attention because of hypertensive crises associated with headache, sweating, and tachycardia. Pheochromocytoma was suspected on the basis of elevated 24-h urinary normetanephrines of 3,418 μg/d, normal value (n.v.) 90–445 (18,662 nmol/d, n.v. 491–2,430) and norepinephrines of 828 μg/d, n.v. 12–86 (4,893 nmol/d, n.v. 71–507). With the integrated use of various radiological and nuclear medicine techniques, a 46-mm retrocardial PGL was diagnosed (Figs. 1 and 2). The patient did not have a family history of pheochromocytoma. Genetic testing excluded RET and SDHx subunit (SDHB, SDHC, and SDHD) mutations. After adequate treatment with doxazosin, cardiac surgery was performed in extracorporeal circulation. The surgical approach was through a median sternotomy. The aorta and the pulmonary artery were divided to access the tumor, which was posterior to the left atrium. Macroscopically, the tumor was encapsulated, and there was a plane of cleavage with the surrounding structures. Complete resection was achieved. Histology confirmed a PGL (chromogranin A and synaptophysin positive at immunohistochemistry; Ki 67, 2%). After surgery, the patient was treated with copious hydration, whereas antihypertensive therapy was withdrawn. Blood pressure was maintained in the normal-low range, and no hypertensive crises were documented. Biochemical evaluations performed 3 months after surgery confirmed the remission of the disease: normetanephrines were 112 μg/d, n.v. 90–445 (611 nmol/d, n.v. 491-2430), and norepinephrines were 20 μg/d, n.v. 12–86 (118 nmol/d, n.v. 71–507).
