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[一名年轻男性患者的原发性转移性肾类癌肿瘤:对神经内分泌肿瘤鉴别诊断的贡献]

[Primary metastatic renal metastatic carcinoid tumor in a young male patient: a contribution to the differential diagnosis of neuroendocrine tumors].

作者信息

Gross M L, Berger I

机构信息

Institut für Pathologie, INF 220/221, 69120, Heidelberg.

出版信息

Pathologe. 2007 Jul;28(4):285-90. doi: 10.1007/s00292-006-0831-4.

Abstract

Primary renal carcinoid tumors originating in normal kidney are extremely rare. We report a case of primary renal carcinoid tumor with an aggressive clinical course and multiple metastases in the paraaortal lymph nodes and the liver as well as a pulmonary metastasis, in a 30-year-old patient. A CT scan of the abdomen revealed a large mass in the right kidney and multiple tumor suspect areas in the liver and paraaortal lymph nodes. The patient did not have clinical manifestations of carcinoid syndrome. Histologically, the tumor was composed of trabecular, solid or anastomosing ribbon-like nests, identical to the features of neuroendocrine tumors from other locations. Immunohistochemical staining was positive for cytokeratin, neurospecific enolase, and chromogranin. Electron microscopically, tonofibrils, primitive desmosomes, and dense-core granules with a neuroendocrine appearance were present. The pathological features of this case are briefly reviewed in comparison with those of previously reportet in terms of certain clinical aspects, prognostic factors and the WHO-classification (2004) of this rare neoplasia.

摘要

起源于正常肾脏的原发性肾类癌极为罕见。我们报告一例30岁患者的原发性肾类癌,其临床病程侵袭性强,出现主动脉旁淋巴结、肝脏多发转移以及肺转移。腹部CT扫描显示右肾有一巨大肿块,肝脏和主动脉旁淋巴结有多个可疑肿瘤区域。该患者没有类癌综合征的临床表现。组织学上,肿瘤由小梁状、实性或吻合的带状巢组成,与其他部位神经内分泌肿瘤的特征相同。免疫组化染色细胞角蛋白、神经特异性烯醇化酶和嗜铬粒蛋白呈阳性。电镜下可见张力原纤维、原始桥粒和具有神经内分泌外观的致密核心颗粒。结合先前报道的病例,从某些临床方面、预后因素以及世界卫生组织(2004年)对这种罕见肿瘤的分类等角度,简要回顾了该病例的病理特征。

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