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Resection of a functioning intrapericardial paraganglioma associatedwith succinate dehydrogenase B mutation

机译:切除功能正常的心包内副神经节瘤琥珀酸脱氢酶B突变

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摘要

Functional paragangliomas are rare neuroendocrine tumours that secrete catecholamines and are infrequently found in the mediastinum. We report a case of a young male with symptoms of catecholamine excess and a personal and family history of the paraganglioma predisposing succinate dehydrogenase subunit B mutation. The lesion had anatomical intrapericardial juxtaposition to important cardiac anatomy and posed the significant challenge of dissection at surgery. The lesion was successfully resected via sternotomy on cardiopulmonary bypass and confirmed histopathologically as paraganglioma. Intrapericardial paraganglioma is rare and treatment is difficult and time critical considering the proximity of cardiac anatomy as well as malignant potential.
机译:功能性神经节旁瘤是罕见的神经内分泌肿瘤,分泌儿茶酚胺,很少见于纵隔。我们报告一例年轻男性,儿茶酚胺过量的症状和副神经节瘤的个人和家族史易诱发琥珀酸脱氢酶亚基B突变。病变与重要的心脏解剖结构在解剖上心包内并置,对手术时的解剖提出了重大挑战。该病灶通过体外循环胸骨切开术成功切除,并在组织病理学上证实为副神经节瘤。心内膜旁神经节瘤很少见,考虑到心脏解剖结构的接近性和恶性潜能,治疗困难且时间紧迫。

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