Retrospective study on mixed neuroendocrine non-neuroendocrine neoplasms from five European centres

查看全文 作　　者：Melissa [1]Frizziero;Xin [2]Wang;Bipasha [3]Chakrabarty;Alexa [4,5]Childs;Tu V [6]Luong;Thomas [7]Walter;Mohid S [8]Khan;Meleri [9]Morgan;Adam [9]Christian;Mona [10]Elshafie;Tahir [11]Shah;Annamaria [12]Minicozzi;Wasat [1]Mansoor;Tim [4,5]Meyer;Angela [1]Lamarca;Richard A [1]Hubner;Juan W [1,13]Valle;Mairéad G [1,13]McNamara

机构地区：[1]Department of Medical Oncology,The Christie NHS Foundation Trust,Manchester M20 4BX,United Kingdom;[2]Department of Analytics and Development,The Christie NHS Foundation Trust,Manchester M20 4BX,United Kingdom;[3]Department of Pathology,The Christie NHS Foundation Trust,Manchester M20 4BX,United Kingdom;[4]Department of Medical Oncology,Royal Free London NHS Foundation Trust,London NW3 2QG,United Kingdom;[5]UCL Cancer Institute,University College London,London WC1E 6AG,United Kingdom;[6]Department of Histopathology,Royal Free London NHS Foundation Trust,London NW3 2QG,United Kingdom;[7]Department of Gastroenterology and Medical Oncology,Edouard Herriot Hospital,Hospices Civils de Lyon,Lyon 69003,France;[8]Department of Gastroenterology,Cardiff and Vale University Health Board,University Hospital of Wales,Cardiff CF14 4XW,United Kingdom;[9]Department of Cellular Pathology,Cardiff and Vale University Health Board,University Hospital of Wales,Cardiff CF14 4XW,United Kingdom;[10]Department of Pathology,University Hospitals Birmingham NHS Foundation Trust,Birmingham B15 2TH,United Kingdom;[11]Department of Hepatology,University Hospitals Birmingham NHS Foundation Trust,Birmingham B15 2TH,United Kingdom;[12]Department of Surgical Oncology,The Christie NHS Foundation Trust,Manchester M20 4BX,United Kingdom;[13]Division of Cancer Sciences,University of Manchester,Manchester M13 9PL,United Kingdom

出　　处：《World Journal of Gastroenterology》索引2019年第25卷第39期,共15页

摘　　要：BACKGROUND Mixed neuroendocrine non-neuroendocrine neoplasm(MiNEN)is a rare diagnosis,mainly encountered in the gastro-entero-pancreatic tract.There is limited knowledge of its epidemiology,prognosis and biology,and the best management for affected patients is still to be defined.AIM To investigate clinical-pathological characteristics,treatment modalities and survival outcomes of a retrospective cohort of patients with a diagnosis of MiNEN.METHODS Consecutive patients with a histologically proven diagnosis of MiNEN were identified at 5 European centres.Patient data were retrospectively collected from medical records.Pathological samples were reviewed to ascertain compliance with the 2017 World Health Organisation definition of MiNEN.Tumour responses to systemic treatment were assessed according to the Response Evaluation Criteria in Solid Tumours 1.1.Kaplan-Meier analysis was applied to estimate survival outcomes.Associations between clinical-pathological characteristics and survival outcomes were explored using Log-rank test for equality of survivors functions(univariate)and Cox-regression analysis(multivariable).RESULTS Sixty-nine consecutive patients identified;Median age at diagnosis:64 years.Males:63.8%.Localised disease(curable):53.6%.Commonest sites of origin:colon-rectum(43.5%)and oesophagus/oesophagogastric junction(15.9%).The neuroendocrine component was;predominant in 58.6%,poorly differentiated in 86.3%,and large cell in 81.25%,of cases analysed.Most distant metastases analysed(73.4%)were occupied only by a poorly differentiated neuroendocrine component.Ninety-four percent of patients with localised disease underwent curative surgery;53%also received perioperative treatment,most often in line with protocols for adenocarcinomas from the same sites of origin.Chemotherapy was offered to most patients(68.1%)with advanced disease,and followed protocols for pure neuroendocrine carcinomas or adenocarcinomas in equal proportion.In localised cases,median recurrence free survival(RFS);14.0 months(95%CI:9.2-24.4),and median overall survival(OS):28.6 months(95%CI:18.3-41.1).On univariate analysis,receipt of perioperative treatment(vs surgery alone)did not improve RFS(P=0.375),or OS(P=0.240).In advanced cases,median progression free survival(PFS);5.6 months(95%CI:4.4-7.4),and median OS;9.0 months(95%CI:5.2-13.4).On univariate analysis,receipt of palliative active treatment(vs best supportive care)prolonged PFS and OS(both,P<0.001).CONCLUSION MiNEN is most commonly driven by a poorly differentiated neuroendocrine component,and has poor prognosis.Advances in its biological understanding are needed to identify effective treatments and improve patient outcomes. 关键词：MIXED NEUROENDOCRINE non-neuroendocrine neoplasm 2017 World Health ORGANISATION classification MIXED adeno-neuroendocrine carcinoma Gastro-enteropancreatic tract DIGESTIVE system NEUROENDOCRINE neoplasms Survival outcomes

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Retrospective study on mixed neuroendocrine non-neuroendocrine neoplasms from five European centres

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