Abstract
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 TP53, BRAF, and KRAS, 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.
Highlights
IntroductionEpithelial neoplasms displaying a coexistence of a neuroendocrine and non-neuroendocrine histology include a wide spectrum of entities composed of a variable proportion of the two histologies (each representing from 1% to 99% of the tumour mass) and have been described in almost all organs [1,2]
Epithelial neoplasms displaying a coexistence of a neuroendocrine and non-neuroendocrine histology include a wide spectrum of entities composed of a variable proportion of the two histologies and have been described in almost all organs [1,2]
This systematic review comprises the largest collection of studies on Mixed neuroendocrine non-neuroendocrine neoplasms (MiNENs) available in the current literature
Summary
Epithelial neoplasms displaying a coexistence of a neuroendocrine and non-neuroendocrine histology include a wide spectrum of entities composed of a variable proportion of the two histologies (each representing from 1% to 99% of the tumour mass) and have been described in almost all organs [1,2]. Neuroendocrine and non-neuroendocrine features coexist at a cellular level (amphicrine tumours) [1,3]. Besides their pathological heterogeneity, over the years, mixed neuroendocrine/non-neuroendocrine neoplasms have been assigned a number of different definitions, with some redundant or only partially overlapping (a comprehensive list of the terms used in the literature has been reported by La Rosa et al [2]), giving rise to a huge inconsistency in published data on these neoplasms. The rationale behind the 30% threshold is that a lesser represented component is unlikely to influence the biological behaviour of the whole neoplasm. This is an arbitrary threshold and not supported by evidence of its clinical relevance or pathogenic significance [1]
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