Siejka Dylan A, Vittorio Alexander F, Thakur Sameer, Burgess John R, Hardikar Ashutosh
Tasmanian Cardiothoracic Unit, Royal Hobart Hospital, Hobart, TAS, Australia.
Department of Endocrinology, Royal Hobart Hospital, Hobart, TAS, Australia.
SAGE Open Med Case Rep. 2019 Apr 1;7:2050313X19839530. doi: 10.1177/2050313X19839530. eCollection 2019.
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.
功能性副神经节瘤是一种罕见的神经内分泌肿瘤,可分泌儿茶酚胺,很少见于纵隔。我们报告一例年轻男性病例,该患者有儿茶酚胺过量症状,且有副神经节瘤易感性琥珀酸脱氢酶亚基B突变的个人及家族史。该病变在心包内与重要心脏结构相邻,给手术切除带来了巨大挑战。通过在体外循环下开胸成功切除该病变,并经组织病理学证实为副神经节瘤。心包内副神经节瘤罕见,鉴于其与心脏结构的毗邻关系以及恶性潜能,治疗困难且时间紧迫。