Scognamiglio Anna, Zappi Arianna, Andrini Elisa, Di Odoardo Adriana, Campana Davide, La Salvia Anna, Lamberti Giuseppe
Department of Medical and Surgical Sciences (DIMEC), Alma Mater Studiorum-University of Bologna, 40126 Bologna, Italy.
Oncology Unit, IRCCS AOUBO, 40138 Bologna, Italy.
J Clin Med. 2025 Aug 13;14(16):5733. doi: 10.3390/jcm14165733.
Neuroendocrine neoplasms (NENs) of the lung are a biologically and clinically diverse group of tumors that includes well-differentiated typical and atypical carcinoids (LNETs), as well as poorly differentiated large-cell neuroendocrine carcinoma and small-cell lung cancer. Despite their relative rarity, the incidence of LNETs is increasing, primarily due to advancements in diagnostic techniques and heightened clinical awareness. While the current World Health Organization (WHO) classification offers a morphological basis for diagnosis and prognosis, particularly for extrapulmonary neuroendocrine neoplasms (ep-NENs), it has limitations in predicting the clinical behavior of pulmonary carcinoids. Recent evidence highlights the inadequacy of traditional criteria in fully capturing the biological complexity and clinical heterogeneity of these tumors. This review explores the evolving landscape of LNETs, focusing on well-differentiated forms and analyzing current classification systems, clinicopathological features, and the emerging role of novel prognostic and predictive biomarkers. Advances in histopathology and molecular profiling have begun to elucidate distinct molecular subsets within carcinoids, offering potential avenues for improved risk stratification and therapeutic decision-making. Although there are limited treatment options for advanced disease, new insights into tumor biology could facilitate the development of personalized therapeutic strategies and pave the way for future innovations in LNET management.
肺神经内分泌肿瘤(NENs)是一组生物学和临床特征各异的肿瘤,包括高分化的典型和非典型类癌(LNETs),以及低分化的大细胞神经内分泌癌和小细胞肺癌。尽管LNETs相对罕见,但其发病率正在上升,主要归因于诊断技术的进步和临床意识的提高。虽然目前世界卫生组织(WHO)的分类为诊断和预后提供了形态学依据,特别是对于肺外神经内分泌肿瘤(ep-NENs),但在预测肺类癌的临床行为方面存在局限性。最近的证据表明,传统标准不足以完全捕捉这些肿瘤的生物学复杂性和临床异质性。本综述探讨了LNETs不断变化的格局,重点关注高分化形式,并分析了当前的分类系统、临床病理特征以及新型预后和预测生物标志物的新兴作用。组织病理学和分子谱分析的进展已开始阐明类癌中的不同分子亚群,为改善风险分层和治疗决策提供了潜在途径。尽管晚期疾病的治疗选择有限,但对肿瘤生物学的新见解可能有助于制定个性化治疗策略,并为LNET管理的未来创新铺平道路。