肝脏原发性多灶囊性印戒神经内分泌肿瘤1例。

Nalini Bansal, Brahmananda Satapathy
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引用次数: 1

摘要

原发性肝印戒神经内分泌肿瘤是一种极为罕见的肿瘤。形态学上,它们与印戒细胞腺癌相似;然而,特殊染色缺乏粘蛋白和神经内分泌标志物的表达有助于诊断这些肿瘤。我们在此报告一例47岁的女性,她表现为双肝叶多发实性和囊性病变,最初影像学提示为胆道囊腺癌。肝脏活检显示有一印戒状肿瘤,伴突触素和泛细胞角蛋白弥漫性表达。该病例随后被诊断为原发性肝印戒神经内分泌肿瘤。患者接受了3个周期的化疗,诊断14个月后保存完好。虽然这是一种极其罕见的实体,但在鉴别诊断以印戒细胞形态为特征的肿瘤时,应考虑其可能性。
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Primary multifocal cystic signet ring neuroendocrine tumor of liver: a case report.

Primary signet ring neuroendocrine tumors of the liver are extremely rare tumors. Morphologically, they mimic signet ring cell adenocarcinomas; however, the absence of mucin by special stains and the expression of neuroendocrine markers help to diagnose these tumors. We herein report a case of a 47-year-old female who presented with multiple solid and cystic lesions in both liver lobes, which were initially suggested to be biliary cystadenocarcinoma on imaging. Liver biopsy of the lesion revealed the presence of a signet ring neoplasm with diffuse expression of synaptophysin and pan-cytokeratin. The case was subsequently diagnosed as a primary hepatic signet ring neuroendocrine tumor. The patient was offered 3 cycles of chemotherapy and is well preserved after 14 months of diagnosis. Although this is an extremely rare entity, its possibility should be considered in the differential diagnosis of neoplasms characterized by signet ring cell morphology.

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