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前纵隔大细胞神经内分泌癌。

Large cell neuroendocrine carcinoma arising from the anterior mediastinum.

机构信息

Diabetes and Endocrinology, Mater Dei Hospital, Msida, Malta

Gastroenterology, Mater Dei Hospital, Msida, Malta.

出版信息

BMJ Case Rep. 2021 May 25;14(5):e240453. doi: 10.1136/bcr-2020-240453.

Abstract

Anterior mediastinal large cell neuroendocrine carcinomas (LCNECs) are extremely rare, extremely aggressive malignancies that carry a dismal prognosis. We discuss a woman aged 60 years who presented with a 2-month history of recurrent severe constant epigastric pain. Abdominal examination revealed massive hepatomegaly and a CT scan of the liver confirmed coarse liver lesions. Histology from a liver biopsy was consistent with a large cell (non-small cell) neuroendocrine carcinoma. A CT scan of the chest showed a large anterior mediastinal mass unrelated to the lung, suggesting that the anterior mediastinum was the primary origin of the tumour. The patient was planned to receive platinum/etoposide chemotherapy for a metastatic mediastinal large cell neuroendocrine carcinoma. Unfortunately, her health deteriorated, and she was unfit to undergo any further treatment. She was treated palliatively and died 2 months after the diagnosis.

摘要

前纵隔大细胞神经内分泌癌(LCNEC)极为罕见,是侵袭性极强的恶性肿瘤,预后极差。我们讨论了一位 60 岁女性,她因反复发作的严重持续上腹痛就诊,病史为 2 个月。腹部检查发现肝肿大,肝脏 CT 扫描证实肝内有粗结节。肝活检组织学检查符合大细胞(非小细胞)神经内分泌癌。胸部 CT 扫描显示前纵隔有一个与肺部无关的大肿块,提示前纵隔是肿瘤的原发部位。该患者计划接受顺铂/依托泊苷化疗治疗纵隔转移大细胞神经内分泌癌。不幸的是,她的健康状况恶化,无法进行进一步治疗。她接受姑息治疗,在诊断后 2 个月死亡。

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本文引用的文献

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Morphologic and molecular classification of lung neuroendocrine neoplasms.肺神经内分泌肿瘤的形态学和分子分类。
Virchows Arch. 2021 Jan;478(1):5-19. doi: 10.1007/s00428-020-03015-z. Epub 2021 Jan 21.
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Neuroendocrine tumors of the thymus and mediastinum.胸腺和纵隔的神经内分泌肿瘤
J Thorac Dis. 2017 Nov;9(Suppl 15):S1448-S1457. doi: 10.21037/jtd.2017.02.02.
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Classification of pulmonary neuroendocrine tumors: new insights.肺神经内分泌肿瘤的分类:新见解
Transl Lung Cancer Res. 2017 Oct;6(5):513-529. doi: 10.21037/tlcr.2017.09.04.
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[Neuroendocrine neoplasms of the mediastinum].[纵隔神经内分泌肿瘤]
Pathologe. 2016 Sep;37(5):434-40. doi: 10.1007/s00292-016-0198-0.

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