嗜鉻細胞瘤是來源于神經嵴的產生兒茶酚胺的腫瘤。原發(fā)性心臟嗜鉻細胞瘤是極為罕見的,全世界目前見于文獻報道的僅為100例左右,北京協(xié)和醫(yī)院心外科去年1月份成功進行了一例心臟嗜鉻細胞瘤切除手術,患者為中年女性,腫瘤同時侵犯左右心房,其MIBG閃爍掃描的結果正常,但奧曲肽顯像結果異常。腫瘤在體外循環(huán)下被成功切除。該病例報告已經發(fā)表在著名心胸外科專業(yè)雜志Annals of Thoracic Surgery上。
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Fig 1. (A) 99m-Technetium-labeled hydrazinonicotinyl-Tyr3-octretide scintiscan demonstrated a focus of intense uptake on right side of heart silhouette. (B) Contrast-enhanced computed tomographic scan illustrated tumor (arrow) overlying interatrial groove. (LA = left atrium; RA = right atrium.)
Fig 2. Coronary angiogram demonstrated a tumor blush with 2 feeding vessels (white arrows) branching from left circumflex artery.
Fig 3. Resected cardiac pheochromocytoma.
Primary Cardiac Pheochromocytoma Involving Both Right and Left Atria
Xingrong Liu, MD, Qi Miao, MD , Heng Zhang, MD, Chaoji Zhang, MD, Lihua Cao, MD, Guotao Ma, MD, Zhengpei Zeng, MD
Pheochromocytomas are catecholamine-producing paragangliomas derived from the neural crest. Primary cardiac pheochromocytoma is extremely rare. We describe a case of cardiac pheochromocytoma involving both right and left atria in a middle-aged woman. Metaiodobenzylguanidine (MIBG) scintiscan results were normal, but results from an octreotide scan were abnormal. The tumor was successfully resected using cardiopulmonary bypass (CPB).