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表达晚期糖基化终末产物受体的肺腺癌合并原发性系统性 AL 淀粉样变性:一例报告及文献复习

Lung adenocarcinoma expressing receptor for advanced glycation end-products with primary systemic AL amyloidosis: a case report and literature review.

作者信息

Okamoto Shouichi, Togo Shinsaku, Nagata Ichiro, Shimizu Kazue, Koinuma Yoshika, Namba Yukiko, Ito Jun, Uekusa Toshimasa, Takahashi Kazuhisa

机构信息

Department of Respiratory Medicine, Juntendo University School of Medicine & Graduate School of Medicine, 2-1-1 Hongo, Bunkyo-ku, Tokyo, 113-8421, Japan.

Research Institute for Diseases of Old Ages, Juntendo University Graduate School of Medicine, Tokyo, Japan.

出版信息

BMC Cancer. 2017 Jan 5;17(1):22. doi: 10.1186/s12885-016-3009-3.

Abstract

BACKGROUND

Receptor for advanced glycation end-products (RAGE), a receptor for amyloids, is constitutively expressed in lungs and generally observed to be downregulated in lung cancer tissues. However, increasing levels of RAGE or serum amyloids is associated with poor outcome in lung cancer patients. We report a rare case of primary systemic amyloid light-chain (AL) amyloidosis in biopsy-proven multiple organs with early-stage non-small cell lung cancer (NSCLC) that displayed strong staining for RAGE in the tumour tissue. Interestingly, compared with randomly selected lung cancer biopsy samples, including all representative histological subtypes of NSCLC and small-cell lung cancer, only the NSCLC in the present case showed strong expression for RAGE that can bind amyloids.

CASE PRESENTATION

A 71-year-old woman was admitted to our hospital for comprehensive investigation of nephrotic syndrome. Computed tomography showed a small nodule in the right upper lung lobe with hilar mediastinal lymph node enlargement. Pathological examination of transbronchial biopsy samples of the nodule yielded a diagnosis of lung adenocarcinoma. Furthermore, the pathological detection of amyloid deposition in biopsy samples of a subcarinal lymph node, gastric and duodenal mucosa, cardiac muscle, and bone marrow led to a diagnosis of primary systemic AL amyloidosis with nephrotic syndrome and cardiomyopathy. In addition, RAGE was detected in lung tumour tissues surrounded by normal lung tissues with amyloid deposition.

CONCLUSION

The RAGE positivity of the lung cancer cells in this case suggests an interaction between amyloid-containing tissues and RAGE-expressing cancer cells. Lung adenocarcinoma with RAGE expression may be a complication of underlying amyloidosis.

摘要

背景

晚期糖基化终末产物受体(RAGE)是一种淀粉样蛋白受体,在肺中组成性表达,且通常在肺癌组织中被观察到表达下调。然而,RAGE或血清淀粉样蛋白水平升高与肺癌患者的不良预后相关。我们报告了一例罕见的原发性系统性轻链(AL)淀粉样变性病例,经活检证实多个器官受累,同时患有早期非小细胞肺癌(NSCLC),其肿瘤组织中RAGE呈强染色。有趣的是,与随机选择的肺癌活检样本(包括NSCLC和小细胞肺癌的所有代表性组织学亚型)相比,仅本病例中的NSCLC显示出能结合淀粉样蛋白的RAGE强表达。

病例介绍

一名71岁女性因肾病综合征的全面检查入院。计算机断层扫描显示右上肺叶有一个小结节,伴有肺门纵隔淋巴结肿大。对该结节的经支气管活检样本进行病理检查,诊断为肺腺癌。此外,在隆突下淋巴结、胃和十二指肠黏膜、心肌及骨髓的活检样本中检测到淀粉样蛋白沉积,从而诊断为原发性系统性AL淀粉样变性伴肾病综合征和心肌病。另外,在有淀粉样蛋白沉积的正常肺组织包围的肺肿瘤组织中检测到了RAGE。

结论

本病例中肺癌细胞的RAGE阳性提示含淀粉样蛋白的组织与表达RAGE的癌细胞之间存在相互作用。表达RAGE的肺腺癌可能是潜在淀粉样变性的一种并发症。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e928/5216608/84a33f15bb8b/12885_2016_3009_Fig1_HTML.jpg

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