Lung neuroendocrine tumors (LNETs) and gastroenteropancreatic neuroendocrine tumors (GEP-NETs) are two distinct types of neuroendocrine tumors (NETs) that have traditionally been treated as a single entity despite originating from different sources. Although they share certain phenotypic characteristics and the expression of neuroendocrine markers, they exhibit differences in their microenvironment, molecular mutations, and responses to various therapeutic regimens. Recent research has explored the genetic alterations in these tumors, revealing dissimilarities in the frequently mutated genes, the role of EGFR in carcinogenesis, the presence of transcription factors, and the immunogenicity of the tumor and its microenvironment. Spread Through Air Spaces (STAS), a phenomenon unique to lung carcinomas, appears to play a crucial role in LNET prognosis. These distinctions are also evident in the cascade response of lung and GI tract neuroendocrine tumors to somatostatin analogs, Peptide Receptor Radionuclide Therapy (PRRT), chemotherapy, and immunotherapy. Identifying similarities and differences between the two groups may improve our understanding of the underlying mechanisms and facilitate the development of more effective treatment strategies.
肺神经内分泌肿瘤(LNETs)与胃肠胰神经内分泌肿瘤(GEP-NETs)是两种不同类型的神经内分泌肿瘤(NETs),尽管起源不同,传统上却被视为同一类疾病。尽管它们具有某些表型特征并表达神经内分泌标志物,但在微环境、分子突变及对不同治疗方案的响应方面存在差异。近期研究探索了这些肿瘤的基因改变,揭示了它们在常见突变基因、EGFR在致癌中的作用、转录因子的存在以及肿瘤及其微环境的免疫原性等方面的不同。气腔播散(STAS)作为肺癌特有的现象,似乎在LNET的预后中起着关键作用。这些差异也体现在肺与胃肠道神经内分泌肿瘤对生长抑素类似物、肽受体放射性核素治疗(PRRT)、化疗及免疫治疗的级联反应中。识别两组肿瘤的共性与差异,可能增进我们对潜在机制的理解,并有助于制定更有效的治疗策略。
Lung NETs and GEPNETs: One Cancer with Different Origins or Two Distinct Cancers?