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Recent Advances in Neuroendocrine Neoplasms Research

A special issue of International Journal of Molecular Sciences (ISSN 1422-0067). This special issue belongs to the section "Molecular Oncology".

Deadline for manuscript submissions: closed (28 February 2022) | Viewed by 8376

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Guest Editor
Department of Abdominal Oncology, Clinical and Experimental Oncology, Istituto Nazionale Tumori di Napoli "G. Pascale" IRCCS, National Cancer Institute, Naples, Italy.

Special Issue Information

Dear Colleagues,

Neuroendocrine Neoplasms (NENs) are rare tumors that arise from the diffuse neuroendocrine system. Although NENs are usually considered rare neoplasms than non-neuroendocrine neoplasms, their incidence and prevalence is increased in the last decades, as reported by epidemiological studies. NENs are also very heterogeneous tumors both biologically and for their clinical behavior. Indeed, the management of these diseases requires a multidisciplinary team consisting of a wide range of members, such as: oncologists, endocrinologists, pathologists, nuclear medicine specialists, radiologists, and molecular biologists. Recently, many efforts have been done to shed light the biological mechanisms involved in the genesis and the evolution of this tumors, in order to provide novel approaches to their treatment. On the other hand, screening strategies and diagnostic tools have continually evolved allowing an early diagnosis and thus improving the clinical outcome. However, many other studies are ongoing and are expected. In this special issue, we will appreciate original studies and review articles that gather research on the novel discoveries about the molecular biology, genetic, epigenetic and signaling pathways of NENs that could offer real progresses in the management of diagnosis and therapeutic approach of NENs patients. The special issue will also encourage papers approaching biomolecular experiments performed as part of clinical studies.

Dr. Salvatore Tafuto
Guest Editor

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Published Papers (3 papers)

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Research

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10 pages, 5130 KiB  
Article
Aberrant Expression of Long Non Coding RNA HOTAIR and De-Regulation of the Paralogous 13 HOX Genes Are Strongly Associated with Aggressive Behavior of Gastro-Entero-Pancreatic Neuroendocrine Tumors
by Annabella Di Mauro, Giosuè Scognamiglio, Gabriella Aquino, Margherita Cerrone, Giuseppina Liguori, Ottavia Clemente, Maurizio Di Bonito, Monica Cantile, Gerardo Botti, Salvatore Tafuto and Fabiana Tatangelo
Int. J. Mol. Sci. 2021, 22(13), 7049; https://doi.org/10.3390/ijms22137049 - 30 Jun 2021
Cited by 8 | Viewed by 2117
Abstract
Gastro-entero-pancreatic neuroendocrine neoplasms (GEP-NENs) are rare diseases occurring in the gastrointestinal tract and pancreas. They are characterized by the loss of epithelial tubular gland elements, and by the increased expression of neuroendocrine markers. GEP-NENs are subdivided into two histo-pathological types, gastro-entero-pancreatic neuroendocrine tumors [...] Read more.
Gastro-entero-pancreatic neuroendocrine neoplasms (GEP-NENs) are rare diseases occurring in the gastrointestinal tract and pancreas. They are characterized by the loss of epithelial tubular gland elements, and by the increased expression of neuroendocrine markers. GEP-NENs are subdivided into two histo-pathological types, gastro-entero-pancreatic neuroendocrine tumors (GEP-NETs) and gastro-entero-pancreatic neuroendocrine carcinomas (GEP-NECs). According to WHO 2017 and 2019 classification criteria are graded and staged in four categories, NET-G1, NET-G2, NET-G3, and NEC-G3. The molecular characterization of these tumors can be fundamental for the identification of new diagnostic, prognostic and predictive biomarkers. The main purpose of this study was to analyze the expression of the paralogous 13 HOX genes, normally involved in embryogenic development and frequently deregulated in human cancers, and of the HOX regulating lncRNA HOTAIR in GEP-NENs. The expression of HOX genes is gradually lost in the transition from GEP NET G1 to NET/NEC G3 tumors, while HOTAIR expression, inversely correlated with HOX genes expression and weakly expressed in low-grade GEP NENs, becomes aberrant in NET G3 and NEC G3 categories. Our data highlights their potential role in the molecular stratification of GEP-NENs by suggesting new prognostic markers and potential therapeutic targets. Full article
(This article belongs to the Special Issue Recent Advances in Neuroendocrine Neoplasms Research)
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Review

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15 pages, 321 KiB  
Review
Multimodal Strategy in Localized Merkel Cell Carcinoma: Where Are We and Where Are We Heading?
by Gianluca Ricco, Elisa Andrini, Giambattista Siepe, Cristina Mosconi, Valentina Ambrosini, Claudio Ricci, Riccardo Casadei, Davide Campana and Giuseppe Lamberti
Int. J. Mol. Sci. 2021, 22(19), 10629; https://doi.org/10.3390/ijms221910629 - 30 Sep 2021
Cited by 4 | Viewed by 2278
Abstract
Merkel cell carcinoma (MCC) is an aggressive neuroendocrine tumor of the skin whose incidence is rising. Multimodal treatment is crucial in the non-metastatic, potentially curable setting. However, the optimal management of patients with non-metastatic MCC is still unclear. In addition, novel insights into [...] Read more.
Merkel cell carcinoma (MCC) is an aggressive neuroendocrine tumor of the skin whose incidence is rising. Multimodal treatment is crucial in the non-metastatic, potentially curable setting. However, the optimal management of patients with non-metastatic MCC is still unclear. In addition, novel insights into tumor biology and newly developed treatments (e.g., immune checkpoint inhibitors) that dramatically improved outcomes in the advanced setting are being investigated in earlier stages with promising results. Nevertheless, the combination of new strategies with consolidated ones needs to be clarified. We reviewed available evidence supporting the current treatment recommendations of localized MCC with a focus on potentially ground-breaking future strategies. Advantages and disadvantages of the different treatment modalities, including surgery, radiotherapy, chemotherapy, and immunotherapy in the non-metastatic setting, are analyzed, as well as those of different treatment modalities (adjuvant as opposed to neoadjuvant). Lastly, we provide an outlook of remarkable ongoing studies and of promising agents and strategies in the treatment of patients with non-metastatic MCC. Full article
(This article belongs to the Special Issue Recent Advances in Neuroendocrine Neoplasms Research)
21 pages, 1154 KiB  
Review
The Role of miRNA in the Pathophysiology of Neuroendocrine Tumors
by Lukas Geisler, Raphael Mohr, Joeri Lambrecht, Jana Knorr, Henning Jann, Sven H. Loosen, Burcin Özdirik, Tom Luedde, Linda Hammerich, Frank Tacke, Alexander Wree, Teresa Hellberg and Christoph Roderburg
Int. J. Mol. Sci. 2021, 22(16), 8569; https://doi.org/10.3390/ijms22168569 - 9 Aug 2021
Cited by 12 | Viewed by 3374
Abstract
Neuroendocrine tumors (NETs) represent a tumor group that is both rare and heterogeneous. Prognosis is largely determined by the tumor grading and the site of the primary tumor and metastases. Despite intensive research efforts, only modest advances in diagnostic and therapeutic approaches have [...] Read more.
Neuroendocrine tumors (NETs) represent a tumor group that is both rare and heterogeneous. Prognosis is largely determined by the tumor grading and the site of the primary tumor and metastases. Despite intensive research efforts, only modest advances in diagnostic and therapeutic approaches have been achieved in recent years. For patients with non-respectable tumor stages, prognosis is poor. In this context, the development of novel diagnostic tools for early detection of NETs and prediction of tumor response to therapy as well as estimation of the overall prognosis would greatly improve the clinical management of NETs. However, identification of novel diagnostic molecules is hampered by an inadequate understanding of the pathophysiology of neuroendocrine malignancies. It has recently been demonstrated that microRNA (miRNA), a family of small RNA molecules with an established role in the pathophysiology of quite different cancer entities, may also play a role as a biomarker. Here, we summarize the available knowledge on the role of miRNAs in the development of NET and highlight their potential use as serum-based biomarkers in the context of this disease. We discuss important challenges currently preventing their use in clinical routine and give an outlook on future directions of miRNA research in NET. Full article
(This article belongs to the Special Issue Recent Advances in Neuroendocrine Neoplasms Research)
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