Pulmonary Large Cell Neuroendocrine Carcinoma.

Pulmonary Large Cell Neuroendocrine Carcinoma.
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DOI:
10.3389/pore.2022.1610730
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发表时间:
2022
影响因子:
2.8
通讯作者:
Lu, Hongyang
Lu, Hongyang
中科院分区:
医学4区
文献类型:
--
作者:
Yang, Lan;Fan, Ying;Lu, Hongyang

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肺大细胞神经内分泌癌(LCNEC)是一种罕见的肺恶性肿瘤亚型。据报道,LCNEC在肺癌中的发生率为0.3%-3%。虽然LCNEC被归类为非小细胞肺癌(NSCLC),但它比其他NSCLC更具侵袭性和恶性,其生物学行为与小细胞肺癌(SCLC)相似。LCNEC患者多为老年吸烟男性,临床表现无特异性。肿瘤的影像学表现多位于肺上叶及周围,纵隔或肺门淋巴结肿大常见。诊断主要依据病理组织学特征和免疫组化(IHC)。特异性神经内分泌标志物如嗜铬粒蛋白A(CgA)、突触素(Syn)和CD 56在LCNEC中常呈弥漫性阳性,发现胰岛素瘤相关蛋白(INSM 1)和Ki-67阳性率高有助于诊断。更多的鉴别诊断也增加了正确诊断LCNEC的难度。近年来兴起的LCNEC分子分型方法可能有助于诊断和后续治疗。本文就LCNEC的流行病学特征、影像学研究、病理学、诊断、治疗及预后作一综述。
Pulmonary large cell neuroendocrine carcinoma (LCNEC) is a rare subtype of malignant pulmonary tumor. The incidence rate of LCNEC was reported to be 0.3%–3% in lung cancers. Although LCNEC is classified as non-small cell lung cancer (NSCLC), it is more aggressive and malignant than other NSCLC, and its biological behavior is similar to that of small cell lung cancer (SCLC). Most of the LCNEC patients are elderly smoking male and the clinical manifestations are not specific. The imaging manifestations of the tumors are often located in the periphery and the upper lobes, and the enlargement of mediastinal or hilar lymph nodes is common. The diagnosis is mainly based on pathology by the histological features and immunohistochemistry (IHC). Specific neuroendocrine markers such as chromogranin A (CgA), synaptophysin (Syn) and CD56 are usually diffusely positive in LCNEC, and found that insulinoma-associated protein (INSM1) and high rate of Ki-67 are helpful for diagnosis. More differential diagnoses also increase the difficulty of correctly diagnosing LCNEC. The rise of LCNEC molecular typing in recent years may be helpful for diagnosis and subsequent treatment. This review focuses on the epidemiological features, imaging studies, pathology, diagnosis, treatment, and prognosis of LCNEC.
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