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[胰腺副神经节瘤转移至肾上腺:一例报告]

[Paraganglioma of the pancreas metastasized to the adrenal gland: a case report].

作者信息

Paik Kwang Yeol

机构信息

Department of Surgery, Kepco Medical Foundation, Hanil General Hospital, Seoul, Korea.

出版信息

Korean J Gastroenterol. 2009 Dec;54(6):409-12. doi: 10.4166/kjg.2009.54.6.409.

Abstract

Paraganglioma is a rare neuroendocrine tumor arising from the neural crest, which includes tissues such as the adrenal medulla, carotid and aortic body, organs of Zuckerkandl, and other unnamed paraganglia. The head, neck, and retroperitoneum are the most common sites for paraganglioma. However, paraganglioma of the pancreas is extremely rare. We report our experience of this rare disease. A 70-year old female patient admitted for a pancreas tail mass detected by computed tomography (CT) scan checked for vague left flank pain. CT with contrast enhancement showed a 4.2-cm heterogeneously enhanced lesion in the tail of the pancreas. A well defined ovoid shape mass in left adrenal gland was suggested adenoma. Distal pancreatectomy with left adrenalectomy was performed. Any lymph node enlargement was not found. Pancreas mass did not invade adjacent organ. Microscopic examination with pancreas and adrenal gland revealed that the cells were arranged in a characteristic Zellballen pattern. Immunohistochemical staining revealed positivity for neuron-specific enolase, chromogranin A, synaptophysin, and S-100 protein. On the basis of these findings, we diagnosed the tumor as a paraganglioma of the pancreas and adrenal gland. We report the first case of pancreas paraganglioma in Korea.

摘要

副神经节瘤是一种罕见的神经内分泌肿瘤,起源于神经嵴,包括肾上腺髓质、颈动脉体和主动脉体、祖克坎德尔器官以及其他未命名的副神经节等组织。头颈部和腹膜后是副神经节瘤最常见的部位。然而,胰腺副神经节瘤极为罕见。我们报告了我们对这种罕见疾病的诊治经验。一名70岁女性患者因计算机断层扫描(CT)检查发现胰腺尾部肿块而入院,该患者因左侧胁腹隐痛就诊。增强CT显示胰腺尾部有一个4.2厘米的不均匀强化病变。左肾上腺一个边界清晰的椭圆形肿块提示为腺瘤。遂行胰体尾切除术及左肾上腺切除术。未发现任何淋巴结肿大。胰腺肿块未侵犯相邻器官。对胰腺和肾上腺进行显微镜检查发现,细胞呈特征性的巢状排列。免疫组化染色显示神经元特异性烯醇化酶、嗜铬粒蛋白A、突触素和S-100蛋白呈阳性。基于这些发现,我们将该肿瘤诊断为胰腺和肾上腺副神经节瘤。我们报告了韩国首例胰腺副神经节瘤病例。

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