Frizziero Melissa, Chakrabarty Bipasha, Nagy Bence, Lamarca Angela, Hubner Richard A, Valle Juan W, McNamara Mairéad G
Department of Medical Oncology, The Christie NHS Foundation Trust, 550 Wilmslow Road, Manchester M20 4BX, UK.
Department of Pathology, The Christie NHS Foundation Trust, 550 Wilmslow Road, Manchester M20 4BX, UK.
J Clin Med. 2020 Jan 19;9(1):273. doi: 10.3390/jcm9010273.
Mixed neuroendocrine non-neuroendocrine neoplasms (MiNENs) represent a rare diagnosis of the gastro-entero-pancreatic tract. Evidence from the current literature regarding their epidemiology, biology, and management is of variable quality and conflicting. Based on available data, the MiNEN has an aggressive biological behaviour, mostly driven by its (often high-grade) neuroendocrine component, and a dismal prognosis. In most cases, the non-neuroendocrine component is of adenocarcinoma histology. Due to limitations in diagnostic methods and poor awareness within the scientific community, the incidence of MiNENs may be underestimated. In the absence of data from clinical trials, MiNENs are commonly treated according to the standard of care for pure neuroendocrine carcinomas or adenocarcinomas from the same sites of origin, based on the assumption of a biological similarity to their pure counterparts. However, little is known about the molecular aberrations of MiNENs, and their pathogenesis remains controversial; molecular/genetic studies conducted so far point towards a common monoclonal origin of the two components. In addition, mutations in tumour-associated genes, including , and , and microsatellite instability have emerged as potential drivers of MiNENs. This systematic review (91 full manuscripts or abstracts in English language) summarises the current reported literature on clinical, pathological, survival, and molecular/genetic data on MiNENs.
混合性神经内分泌-非神经内分泌肿瘤（MiNENs）是一种罕见的胃肠道和胰腺肿瘤诊断。目前文献中关于其流行病学、生物学和治疗的证据质量参差不齐且相互矛盾。根据现有数据，MiNENs具有侵袭性生物学行为，主要由其（通常为高级别）神经内分泌成分驱动，预后不佳。在大多数情况下，非神经内分泌成分的组织学类型为腺癌。由于诊断方法的局限性以及科学界认识不足，MiNENs的发病率可能被低估。在缺乏临床试验数据的情况下，基于与纯神经内分泌癌或来自相同起源部位的腺癌生物学相似性的假设，MiNENs通常按照这些纯肿瘤的标准治疗方案进行治疗。然而，关于MiNENs的分子异常知之甚少，其发病机制仍存在争议；迄今为止进行的分子/遗传学研究表明这两种成分具有共同的单克隆起源。此外，包括 、 和 在内的肿瘤相关基因突变以及微卫星不稳定性已成为MiNENs的潜在驱动因素。本系统综述（91篇英文全文手稿或摘要）总结了目前已报道的关于MiNENs临床、病理、生存以及分子/遗传学数据的文献。
