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肝脏原发性无功能副神经节瘤:一种罕见于不寻常部位的肿瘤。

Primary non-functioning paraganglioma of liver: a rare tumour at an unusual location.

作者信息

Khan Muhammad Rizwan, Raza Rushna, Jabbar Abdul, Ahmed Arsalan

机构信息

Department of Surgery, Aga Khan University & Hospital, Karachi.

出版信息

J Pak Med Assoc. 2011 Aug;61(8):814-6.

Abstract

Pheochromocytomas are rare tumours, 22% of which are extra-adrenal and are known as paragangliomas. We report a case of a young male with non-functioning paraganglioma of the liver; a very uncommon primary site. A CT scan with contrast showed a huge, highly vascular mass lesion in the right lobe of the liver and a core biopsy confirmed it to be a paraganglioma. Whole body study with iodine-131-meta-iodobenzylguanidine (MIBG) tracer highlighted the liver tumour but was negative for any other active lesions depicting that the tumour was confined to the liver. The lesion was completely resected by extended right hepatectomy and the patient had an uneventful recovery. There is no evidence of disease recurrence at 3 years of follow up.

摘要

嗜铬细胞瘤是罕见肿瘤,其中22%为肾上腺外肿瘤,称为副神经节瘤。我们报告一例年轻男性患有无功能肝脏副神经节瘤的病例,肝脏是非常罕见的原发部位。增强CT扫描显示肝脏右叶有一个巨大的、血供丰富的肿块病变,核心活检证实为副神经节瘤。使用碘-131-间碘苄胍(MIBG)示踪剂进行的全身检查突出显示了肝脏肿瘤,但其他任何活性病变均为阴性,表明肿瘤局限于肝脏。通过扩大右肝切除术将病变完全切除,患者恢复顺利。随访3年无疾病复发迹象。

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