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      Neuroendocrine Carcinomas of the Gastroenteropancreatic System: A Comprehensive Review

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          To date, empirical literature has generally been considered lacking in relation to neuroendocrine carcinomas (NECs), the highly malignant subgroup of neuroendocrine neoplasms. NECs are often found in the lungs or the gastroenteropancreatic (GEP) system and can be of small or large cell type. Concentrating on GEP-NECs, we can conclude that survival times are poor, with a median of only 4–16 months depending on disease stage and primary site. Further, this aggressive disease appears to be on the rise, with incidence numbers increasing while survival times are stagnant. Treatment strategies concerning surgery are often undecided and second-line chemotherapy is not yet established. After an analysis of over 2600 articles, we can conclude that there is indeed more empirical literature concerning GEP-NECs available than previously assumed. This unique review is based on 333 selected articles and contains detailed information concerning all aspects of GEP-NECs. Namely, the classification, histology, genetic abnormalities, epidemiology, origin, biochemistry, imaging, treatment and survival of GEP-NECs are described. Also, organ-specific summaries with more detail in relation to disease presentation, diagnosis, treatment and survival are presented. Finally, key points are discussed with directions for future research priorities.

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          Most cited references 277

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          Predictive and prognostic factors for treatment and survival in 305 patients with advanced gastrointestinal neuroendocrine carcinoma (WHO G3): the NORDIC NEC study.

          As studies on gastrointestinal neuroendocrine carcinoma (WHO G3) (GI-NEC) are limited, we reviewed clinical data to identify predictive and prognostic markers for advanced GI-NEC patients. Data from advanced GI-NEC patients diagnosed 2000-2009 were retrospectively registered at 12 Nordic hospitals. The median survival was 11 months in 252 patients given palliative chemotherapy and 1 month in 53 patients receiving best supportive care (BSC) only. The response rate to first-line chemotherapy was 31% and 33% had stable disease. Ki-67<55% was by receiver operating characteristic analysis the best cut-off value concerning correlation to the response rate. Patients with Ki-67<55% had a lower response rate (15% versus 42%, P<0.001), but better survival than patients with Ki-67≥55% (14 versus 10 months, P<0.001). Platinum schedule did not affect the response rate or survival. The most important negative prognostic factors for survival were poor performance status (PS), primary colorectal tumors and elevated platelets or lactate dehydrogenase (LDH) levels. Advanced GI-NEC patients should be considered for chemotherapy treatment without delay.PS, colorectal primary and elevated platelets and LDH levels were prognostic factors for survival. Patients with Ki-67<55% were less responsive to platinum-based chemotherapy, but had a longer survival. Our data indicate that it may not be correct to consider all GI-NEC as one single disease entity.
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            TNM staging of midgut and hindgut (neuro) endocrine tumors: a consensus proposal including a grading system.

            Criteria for the staging and grading of neuroendocrine tumors (NETs) of midgut and hindgut origin were established at the second Consensus Conference in Frascati (Rome) organized by the European Neuroendocrine Tumor Society (ENETS). The proposed tumor-node-metastasis (TNM) classifications are based on the recently published ENETS Guidelines for the Diagnosis and Treatment of gastroenteropancreatic NETs and follow our previous proposal for foregut tumors. The new TNM classifications for NETs of the ileum, appendix, colon, and rectum, and the grading system were designed, discussed, and consensually approved by all conference participants. These proposals need to be validated and are meant to help clinicians in the stratification, treatment and follow-up of patients.
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              ENETS Consensus Guidelines for the Management of Patients with Liver and Other Distant Metastases from Neuroendocrine Neoplasms of Foregut, Midgut, Hindgut, and Unknown Primary


                Author and article information

                Role: Academic Editor
                Diagnostics (Basel)
                Diagnostics (Basel)
                08 April 2015
                June 2015
                : 5
                : 2
                : 119-176
                [1 ]Neuroendocrine Tumor Centre of Excellence, Rigshospitalet, University of Copenhagen, 2100 Copenhagen, Denmark; E-Mails: seppo.langer@ 123456regionh.dk (S.W.L.); ingridho@ 123456sund.ku.dk (I.H.O.); birgitte.federspiel@ 123456regionh.dk (B.F.); andreas.kjaer@ 123456regionh.dk (A.K.); ulrich.peter.knigge@ 123456regionh.dk (U.K.)
                [2 ]Department of Gastrointestinal Surgery C, Rigshospitalet, University of Copenhagen, 2100 Copenhagen, Denmark
                [3 ]Department of Oncology, Rigshospitalet, University of Copenhagen, 2100 Copenhagen, Denmark
                [4 ]Cluster for Molecular Imaging, Faculty of Health Sciences, University of Copenhagen, 2100 Copenhagen, Denmark
                [5 ]Department of Pathology, Rigshospitalet, University of Copenhagen, 2100 Copenhagen, Denmark
                [6 ]Department of Clinical Physiology, Nuclear Medicine and PET, Rigshospitalet, University of Copenhagen, 2100 Copenhagen, Denmark
                [7 ]Deparment of Endocrinology, Rigshospitalet, University of Copenhagen, 2100 Copenhagen, Denmark
                Author notes
                [* ]Author to whom correspondence should be addressed; E-Mail: ilett@ 123456sund.ku.dk ; Tel.: +45-3023-4132; Fax: +45-3545-2183.
                © 2015 by the authors; licensee MDPI, Basel, Switzerland.

                This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution license ( http://creativecommons.org/licenses/by/4.0/).



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