Department of Medical Oncology, Asociatia Oncohelp, 300239 Timisoara, Romania.
Department of Oncology, University of Medicine and Pharmacy "Victor Babes", 300041 Timisoara, Romania.
Curr Oncol. 2023 Jul 27;30(8):7218-7228. doi: 10.3390/curroncol30080523.
Large cell neuroendocrine carcinoma of the lung (LCNEC) is currently classified as a rare lung cancer subtype, but given the high incidence of lung cancer, the overall number of cases is considerable. The pathologic diagnosis of LCNEC is mainly based on the microscopic appearance of the tumor cells, the mitotic rate, the amount of intra-tumoral necrosis, and the presence of positive neuroendocrine markers identified by immunohistochemistry. Recently, a subdivision into two main categories was proposed based on mutation signatures involving the RB1, TP53, KRAS, and STK11/LKB1 genes, into SCLC-like (small cell lung cancer-like) and NSCLC-like (non-small cell lung cancer-like) LCNEC. In terms of treatment, surgery is still the best option for resectable, stage I-IIIA cases. Chemotherapy and radiotherapy have conflicting evidence. Etoposide/platinum remains the standard chemotherapy regimen. However, based on the newly proposed LCNEC subtypes, some retrospective series report better outcomes using a pathology-driven chemotherapy approach. Encouraging outcomes have also been reported for immunotherapy and targeted therapy, but the real impact of these strategies is still being determined in the absence of adequate prospective clinical trials. The current paper scrutinized the epidemiology, reviewed the reliability of pathologic diagnosis, discussed the need for molecular subtyping, and reviewed the heterogeneity of treatment algorithms in LCNEC.
肺大细胞神经内分泌癌（LCNEC）目前被归类为一种罕见的肺癌亚型，但鉴于肺癌的高发病率，总的病例数量相当可观。LCNEC 的病理诊断主要基于肿瘤细胞的微观外观、有丝分裂率、肿瘤内坏死的程度以及免疫组织化学鉴定的阳性神经内分泌标志物。最近，根据涉及 RB1、TP53、KRAS 和 STK11/LKB1 基因的突变特征，提出了一种将其分为两个主要类别的细分方法，即 SCLC 样（小细胞肺癌样）和 NSCLC 样（非小细胞肺癌样）LCNEC。在治疗方面，手术仍然是可切除的 I 期-IIIA 期病例的最佳选择。化疗和放疗的证据相互矛盾。依托泊苷/铂类仍然是标准的化疗方案。然而，基于新提出的 LCNEC 亚型，一些回顾性系列报告称，采用基于病理学的化疗方法可获得更好的结果。免疫治疗和靶向治疗也取得了令人鼓舞的结果，但在缺乏充分的前瞻性临床试验的情况下，这些策略的实际影响仍在确定之中。本文详细研究了 LCNEC 的流行病学，审查了病理诊断的可靠性，讨论了进行分子亚型分类的必要性，并回顾了 LCNEC 治疗方案的异质性。
