{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,20]],"date-time":"2026-07-20T14:20:43Z","timestamp":1784557243395,"version":"3.55.0"},"reference-count":55,"publisher":"Bioscientifica","issue":"8","content-domain":{"domain":["erc.bioscientifica.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2024,5,22]]},"abstract":"\n Graphical abstract<\/jats:title>\n \n \n <\/jats:p>\n <\/jats:sec>\n \n Abstract<\/jats:title>\n The 5th edition of the World Health Organization (WHO) classification of neuroendocrine neoplasms (NENs) is built to achieve a uniform terminology and to define a similar diagnostic scheme across different anatomic locations. Since the 4th edition, a chapter discussing NENs in nonneuroendocrine organs has been introduced, which proposes a binary system for classification segregating well-differentiated neoplasms, termed neuroendocrine tumors (NETs), and poorly differentiated neoplasms, termed neuroendocrine carcinomas (NECs). A grading system for NETs is based on mitotic index and\/or Ki-67 index and\/or necrosis, depending on the different locations. Although this approach has been already well established in the digestive system, it modifies and homogenizes the classification of NENs in the urinary tract, in female genital organs, and in the male genital system. In the lung and thymus, the double terminology of carcinoid\/NET, already introduced in the 5th edition of the WHO classification of thoracic tumors, is endorsed. This approach undoubtedly helps the multidisciplinary approach for the diagnosis and clinical management of patients affected by these neoplasms, without losing site-specific characteristics that influence the clinical and biological behavior of tumors in different anatomical sites. Other major advances of the new WHO scheme are the homogenization of epidemiological data and the correct integration of data from prospective future studies aimed at the definition of molecular profiles and at the identification of tumor type-specific and patient-specific therapeutic approaches.<\/jats:p>\n <\/jats:sec>","DOI":"10.1530\/erc-24-0004","type":"journal-article","created":{"date-parts":[[2024,5,22]],"date-time":"2024-05-22T13:52:11Z","timestamp":1716385931000},"update-policy":"https:\/\/doi.org\/10.1530\/crossmarkpolicy-8","source":"Crossref","is-referenced-by-count":4,"title":["Changes in categorization or nomenclature within neuroendocrine tumors"],"prefix":"10.1530","volume":"31","author":[{"given":"Giulia","family":"Vocino Trucco","sequence":"first","affiliation":[{"name":"Pathology Unit, SS. Annunziata Hospital, Savigliano, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0719-105X","authenticated-orcid":true,"given":"Marco","family":"Volante","sequence":"additional","affiliation":[{"name":"Department of Oncology, University of Turin, Orbassano, Turin, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"416","reference":[{"key":"bib1","series-title":"Cancer","first-page":"345","article-title":"Molecular profile of pancreatic neuroendocrine neoplasms (PanNENs): opportunities for personalized therapies","volume":"127","author":"Arakelyan","year":"2021","unstructured":"Arakelyan JZohrabyan D & Philip PA2021Molecular profile of pancreatic neuroendocrine neoplasms (PanNENs): opportunities for personalized therapies. Cancer127345\u2013353. (https:\/\/doi.org\/10.1002\/cncr.33354)"},{"key":"bib2","series-title":"Endocrine Pathology","first-page":"433","article-title":"Middle ear \u201cadenoma\u201d: a neuroendocrine tumor with predominant L cell differentiation","volume":"32","author":"Asa","year":"2021a","unstructured":"Asa SLArkun KTischler ASQamar ADeng FMPerez-Ordonez BWeinreb IBishop JAWenig BM & Mete O2021aMiddle ear \u201cadenoma\u201d: a neuroendocrine tumor with predominant L cell differentiation. Endocrine Pathology32433\u2013441. (https:\/\/doi.org\/10.1007\/s12022-021-09684-z)"},{"key":"bib3","series-title":"Endocrine Pathology","first-page":"169","article-title":"Molecular pathology of well-differentiated gastro-entero-pancreatic neuroendocrine tumors","volume":"32","author":"Asa","year":"2021b","unstructured":"Asa SLLa Rosa SBasturk OAdsay VMinnetti M & Grossman AB2021bMolecular pathology of well-differentiated gastro-entero-pancreatic neuroendocrine tumors. Endocrine Pathology32169\u2013191. (https:\/\/doi.org\/10.1007\/s12022-021-09662-5)"},{"key":"bib4","series-title":"Head and Neck Pathology","first-page":"375","article-title":"Neuroendocrine neoplasms of the larynx: a clinicopathologic analysis of 27 neuroendocrine tumors and neuroendocrine carcinomas","volume":"16","author":"Bal","year":"2022","unstructured":"Bal MSharma ARane SUMittal NChaukar DPrabhash K & Patil A2022Neuroendocrine neoplasms of the larynx: a clinicopathologic analysis of 27 neuroendocrine tumors and neuroendocrine carcinomas. Head and Neck Pathology16375\u2013387. (https:\/\/doi.org\/10.1007\/s12105-021-01367-9)"},{"key":"bib5","series-title":"Virchows Archiv","first-page":"129","article-title":"Recent advances and conceptual changes in the classification of neuroendocrine tumors of the thymus","volume":"478","author":"Bohnenberger","year":"2021","unstructured":"Bohnenberger H & Str\u00f6bel P2021Recent advances and conceptual changes in the classification of neuroendocrine tumors of the thymus. Virchows Archiv478129\u2013135. (https:\/\/doi.org\/10.1007\/s00428-021-03037-1)"},{"key":"bib6","series-title":"Cancers (Basel)","first-page":"1461","article-title":"PDX1 DNA methylation distinguishes two subtypes of pancreatic neuroendocrine neoplasms with a different prognosis","volume":"12","author":"Boons","year":"2020","unstructured":"Boons GVandamme TIbrahim JRoeyen GDriessen APeeters DLawrence BPrint CPeeters MVan Camp G, et al.2020PDX1 DNA methylation distinguishes two subtypes of pancreatic neuroendocrine neoplasms with a different prognosis. Cancers (Basel)121461. (https:\/\/doi.org\/10.3390\/cancers12061461)"},{"key":"bib7","series-title":"Current Oncology","first-page":"1381","article-title":"Prospective evaluation of MGMT-promoter methylation status and correlations with outcomes to temozolomide-based chemotherapy in well-differentiated neuroendocrine tumors","volume":"30","author":"Brighi","year":"2023","unstructured":"Brighi NLamberti GAndrini EMosconi CManuzzi LDonati GLisotti A & Campana D2023Prospective evaluation of MGMT-promoter methylation status and correlations with outcomes to temozolomide-based chemotherapy in well-differentiated neuroendocrine tumors. Current Oncology301381\u20131394. (https:\/\/doi.org\/10.3390\/curroncol30020106)"},{"key":"bib8","series-title":"Nature Medicine","first-page":"1260","article-title":"Enhancer signatures stratify and predict outcomes of non-functional pancreatic neuroendocrine tumors","volume":"25","author":"Cejas","year":"2019","unstructured":"Cejas PDrier YDreijerink KMABrosens LAADeshpande VEpstein CBConemans EBMorsink FHMGraham MKValk GD, et al.2019Enhancer signatures stratify and predict outcomes of non-functional pancreatic neuroendocrine tumors. Nature Medicine251260\u20131265. (https:\/\/doi.org\/10.1038\/s41591-019-0493-4)"},{"key":"bib9","series-title":"Histopathology","first-page":"324","article-title":"Lung carcinoid tumours: histology and Ki-67, the eternal rivalry","volume":"82","author":"Centonze","year":"2023","unstructured":"Centonze GMaisonneuve PSimbolo MLagano VGrillo FFabbri APrinzi NGarzone GFilugelli MPardo C, et al.2023Lung carcinoid tumours: histology and Ki-67, the eternal rivalry. Histopathology82324\u2013339. (https:\/\/doi.org\/10.1111\/his.14819)"},{"key":"bib10","series-title":"Nature Communications","first-page":"4158","article-title":"ATRX, DAXX or MEN1 mutant pancreatic neuroendocrine tumors are a distinct alpha-cell signature subgroup","volume":"9","author":"Chan","year":"2018","unstructured":"Chan CSLaddha SVLewis PWKoletsky MSRobzyk KDa Silva ETorres PJUntch BRLi JBose P, et al.2018ATRX, DAXX or MEN1 mutant pancreatic neuroendocrine tumors are a distinct alpha-cell signature subgroup. Nature Communications94158. (https:\/\/doi.org\/10.1038\/s41467-018-06498-2)"},{"key":"bib11","series-title":"Asian Pacific Journal of Cancer Prevention","first-page":"113","article-title":"Integrating a 92-gene expression analysis for the management of neuroendocrine tumors of unknown primary","volume":"20","author":"Chauhan","year":"2019","unstructured":"Chauhan AFarooqui ZSilva SRMurray AHodges KBYu QMyint ZWRaajesekar AKWeiss HArnold S, et al.2019Integrating a 92-gene expression analysis for the management of neuroendocrine tumors of unknown primary. Asian Pacific Journal of Cancer Prevention20113\u2013116. (https:\/\/doi.org\/10.31557\/APJCP.2019.20.1.113)"},{"key":"bib12","series-title":"Histopathology","first-page":"439","article-title":"What's new in WHO fifth edition\u2009-\u2009urinary tract","volume":"81","author":"Comp\u00e9rat","year":"2022","unstructured":"Comp\u00e9rat EAmin MBBerney DMCree IMenon SMoch HNetto GJRao VRaspollini MRRubin MA, et al.2022What's new in WHO fifth edition\u2009-\u2009urinary tract. Histopathology81439\u2013446. (https:\/\/doi.org\/10.1111\/his.14764)"},{"key":"bib13","series-title":"Endocrine-Related Cancer","first-page":"e230203","article-title":"Transformation of G1-G2 neuroendocrine tumors to neuroendocrine carcinomas following peptide receptor radionuclide therapy","volume":"31","author":"Cordero-Hernandez","year":"2024","unstructured":"Cordero-Hernandez ISRoss ACDasari AHalperin DMChasen B & Yao JC2024Transformation of G1-G2 neuroendocrine tumors to neuroendocrine carcinomas following peptide receptor radionuclide therapy. Endocrine-Related Cancer31e230203. (https:\/\/doi.org\/10.1530\/ERC-23-0203)"},{"key":"bib14","series-title":"Modern Pathology","first-page":"1829","article-title":"RB1-deficient squamous cell carcinoma: the proposed source of combined Merkel cell carcinoma","volume":"35","author":"DeCoste","year":"2022","unstructured":"DeCoste RCWalsh NMGaston DLy TYPasternak SCutler SNightingale M & Carter MD2022RB1-deficient squamous cell carcinoma: the proposed source of combined Merkel cell carcinoma. Modern Pathology351829\u20131836. (https:\/\/doi.org\/10.1038\/s41379-022-01151-2)"},{"key":"bib15","series-title":"Journal of Thoracic Oncology","first-page":"752","article-title":"New insights into the molecular characteristics of pulmonary carcinoids and large cell neuroendocrine carcinomas, and the impact on their clinical management","volume":"13","author":"Derks","year":"2018","unstructured":"Derks JLLeblay NLantuejoul SDingemans ACSpeel EM & Fernandez-Cuesta L2018New insights into the molecular characteristics of pulmonary carcinoids and large cell neuroendocrine carcinomas, and the impact on their clinical management. Journal of Thoracic Oncology13752\u2013766. (https:\/\/doi.org\/10.1016\/j.jtho.2018.02.002)"},{"key":"bib16","series-title":"Endocrine Pathology","first-page":"64","article-title":"Overview of the 2022 WHO classification of parathyroid tumors","volume":"33","author":"Erickson","year":"2022","unstructured":"Erickson LAMete OJuhlin CCPerren A & Gill AJ2022Overview of the 2022 WHO classification of parathyroid tumors. Endocrine Pathology3364\u201389. (https:\/\/doi.org\/10.1007\/s12022-022-09709-1)"},{"key":"bib17","series-title":"Endocrine Pathology","first-page":"437","article-title":"The driver role of pathologists in endocrine oncology: what clinicians seek in pathology reports","volume":"3","author":"Ezzat","year":"2023","unstructured":"Ezzat Sde Herder WWVolante M & Grossman A2023The driver role of pathologists in endocrine oncology: what clinicians seek in pathology reports. Endocrine Pathology34437\u2013454. (https:\/\/doi.org\/10.1007\/s12022-023-09768-y)"},{"key":"bib18","series-title":"American Journal of Surgical Pathology","first-page":"25","article-title":"A clinicopathologic and molecular analysis of fumarate hydratase-deficient pheochromocytoma and paraganglioma","volume":"47","author":"Fuchs","year":"2023","unstructured":"Fuchs TLLuxford CClarkson ASheen ASioson LElston MCroxson MSDwight TBenn DETacon L, et al.2023A clinicopathologic and molecular analysis of fumarate hydratase-deficient pheochromocytoma and paraganglioma. American Journal of Surgical Pathology4725\u201336. (https:\/\/doi.org\/10.1097\/PAS.0000000000001945)"},{"key":"bib19","series-title":"Cancer Cell","first-page":"346","article-title":"Patterns of transcription factor programs and immune pathway activation define four major subtypes of SCLC with distinct therapeutic vulnerabilities","volume":"39","author":"Gay","year":"2021","unstructured":"Gay CMStewart CAPark EMDiao LGroves SMHeeke SNabet BYFujimoto JSolis LMLu W, et al.2021Patterns of transcription factor programs and immune pathway activation define four major subtypes of SCLC with distinct therapeutic vulnerabilities. Cancer Cell39346\u2013360.e7. 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(https:\/\/doi.org\/10.1038\/s41467-018-03099-x)"},{"key":"bib21","series-title":"American Journal of Surgical Pathology","first-page":"35","article-title":"Parafibromin-deficient (HPT-JT type, CDC73 mutated) parathyroid tumors demonstrate distinctive morphologic features","volume":"43","author":"Gill","year":"2019","unstructured":"Gill AJLim GCheung VKYAndrici JPerry-Keene JLPaik JSioson LClarkson ASheen ALuxford C, et al.2019Parafibromin-deficient (HPT-JT type, CDC73 mutated) parathyroid tumors demonstrate distinctive morphologic features. American Journal of Surgical Pathology4335\u201346. (https:\/\/doi.org\/10.1097\/PAS.0000000000001017)"},{"key":"bib22","series-title":"Genes and Development","first-page":"915","article-title":"POU2F3 is a master regulator of a tuft cell-like variant of small cell lung cancer","volume":"32","author":"Huang","year":"2018","unstructured":"Huang YHKlingbeil OHe XYWu XSArun GLu BSomerville TDDMilazzo JPWilkinson JEDemerdash OE, et al.2018POU2F3 is a master regulator of a tuft cell-like variant of small cell lung cancer. Genes and Development32915\u2013928. (https:\/\/doi.org\/10.1101\/gad.314815.118)"},{"key":"bib23","series-title":"Journal of Clinical Endocrinology and Metabolism","first-page":"1132","article-title":"TNM staging and overall survival in patients with pheochromocytoma and sympathetic paraganglioma","volume":"108","author":"Jimenez","year":"2023","unstructured":"Jimenez CMa JRoman Gonzalez AVarghese JZhang MPerrier NHabra MAGraham P & Waguespack SG2023TNM staging and overall survival in patients with pheochromocytoma and sympathetic paraganglioma. Journal of Clinical Endocrinology and Metabolism1081132\u20131142. (https:\/\/doi.org\/10.1210\/clinem\/dgac677)"},{"key":"bib24","series-title":"Cancers (Basel)","first-page":"2210","article-title":"Metastatic neuroendocrine neoplasms of unknown primary: clues from pathology workup","volume":"14","author":"Juhlin","year":"2022","unstructured":"Juhlin CCZedenius J & H\u00f6\u00f6g A2022Metastatic neuroendocrine neoplasms of unknown primary: clues from pathology workup. Cancers (Basel)142210. (https:\/\/doi.org\/10.3390\/cancers14092210)"},{"key":"bib25","series-title":"Endocrine Pathology","first-page":"1","article-title":"Update on C-cell neuroendocrine neoplasm: prognostic and predictive histopathologic and molecular features of medullary thyroid carcinoma","volume":"34","author":"Jung","year":"2023","unstructured":"Jung CKAgarwal SHang JFLim DJBychkov A & Mete O2023Update on C-cell neuroendocrine neoplasm: prognostic and predictive histopathologic and molecular features of medullary thyroid carcinoma. Endocrine Pathology341\u201322. (https:\/\/doi.org\/10.1007\/s12022-023-09753-5)"},{"key":"bib26","series-title":"Pathology International","first-page":"488","article-title":"Neuroendocrine tumor G3 of bronchopulmonary origin and its classification","volume":"72","author":"Kasajima","year":"2022","unstructured":"Kasajima A & Kl\u00f6ppel G2022Neuroendocrine tumor G3 of bronchopulmonary origin and its classification. Pathology International72488\u2013495. (https:\/\/doi.org\/10.1111\/pin.13266)"},{"key":"bib27","series-title":"Modern Pathology","first-page":"44","article-title":"A 92-gene cancer classifier predicts the site of origin for neuroendocrine tumors","volume":"27","author":"Kerr","year":"2014","unstructured":"Kerr SESchnabel CASullivan PSZhang YHuang VJErlander MGBrachtel EF & Dry SM2014A 92-gene cancer classifier predicts the site of origin for neuroendocrine tumors. Modern Pathology2744\u201354. (https:\/\/doi.org\/10.1038\/modpathol.2013.105)"},{"key":"bib28","series-title":"Endocrine Pathology","first-page":"284","article-title":"Mixed neuroendocrine-nonneuroendocrine neoplasms (MiNENs): unifying the concept of a heterogeneous group of neoplasms","volume":"27","author":"La Rosa","year":"2016","unstructured":"La Rosa SSessa F & Uccella S2016Mixed neuroendocrine-nonneuroendocrine neoplasms (MiNENs): unifying the concept of a heterogeneous group of neoplasms. Endocrine Pathology27284\u2013311. (https:\/\/doi.org\/10.1007\/s12022-016-9432-9)"},{"key":"bib29","series-title":"Oncotarget","first-page":"73745","article-title":"Reciprocal expression of INSM1 and YAP1 defines subgroups in small cell lung cancer","volume":"8","author":"McColl","year":"2017","unstructured":"McColl KWildey GSakre NLipka MBBehtaj MKresak AChen YYang MVelcheti VFu P, et al.2017Reciprocal expression of INSM1 and YAP1 defines subgroups in small cell lung cancer. Oncotarget873745\u201373756. (https:\/\/doi.org\/10.18632\/oncotarget.20572)"},{"key":"bib30","series-title":"Endocrine Pathology","first-page":"90","article-title":"Overview of the 2022 WHO classification of paragangliomas and pheochromocytomas","volume":"33","author":"Mete","year":"2022","unstructured":"Mete OAsa SLGill AJKimura Nde Krijger RR & Tischler A2022Overview of the 2022 WHO classification of paragangliomas and pheochromocytomas. Endocrine Pathology3390\u2013114. (https:\/\/doi.org\/10.1007\/s12022-022-09704-6)"},{"key":"bib31","series-title":"Virchows Archiv","first-page":"839","article-title":"Neuroendocrine neoplasms of the breast: diagnostic agreement and impact on outcome","volume":"481","author":"Metovic","year":"2022","unstructured":"Metovic JCascardi EUccella SMaragliano RQuerzoli GOsella-Abate SPittaro ALa Rosa SBogina GCassoni P, et al.2022Neuroendocrine neoplasms of the breast: diagnostic agreement and impact on outcome. Virchows Archiv481839\u2013846. (https:\/\/doi.org\/10.1007\/s00428-022-03426-0)"},{"key":"bib32","series-title":"Human Pathology","first-page":"123","article-title":"The 2022 revision of the World Health Organization classification of tumors of the urinary system and male genital organs: advances and challenges","volume":"136","author":"Mohanty","year":"2023","unstructured":"Mohanty SKLobo A & Cheng L2023The 2022 revision of the World Health Organization classification of tumors of the urinary system and male genital organs: advances and challenges. Human Pathology136123\u2013143. (https:\/\/doi.org\/10.1016\/j.humpath.2022.08.006)"},{"key":"bib33","series-title":"Journal of Thoracic Oncology","first-page":"362","article-title":"The 2021 WHO classification of lung tumors: impact of advances since 2015","volume":"17","author":"Nicholson","year":"2022","unstructured":"Nicholson AGTsao MSBeasley MBBorczuk ACBrambilla ECooper WADacic SJain DKerr KMLantuejoul S, et al.2022The 2021 WHO classification of lung tumors: impact of advances since 2015. Journal of Thoracic Oncology17362\u2013387. (https:\/\/doi.org\/10.1016\/j.jtho.2021.11.003)"},{"key":"bib34","series-title":"Modern Pathology","first-page":"807","article-title":"SDHB\/SDHA immunohistochemistry in pheochromocytomas and paragangliomas: a multicenter interobserver variation analysis using virtual microscopy: a multinational study of the European Network for the Study of Adrenal Tumors (ENS@T)","volume":"28","author":"Papathomas","year":"2015","unstructured":"Papathomas TGOudijk LPersu AGill AJvan Nederveen FTischler ASTissier FVolante MMatias-Guiu XSmid M, et al.2015SDHB\/SDHA immunohistochemistry in pheochromocytomas and paragangliomas: a multicenter interobserver variation analysis using virtual microscopy: a multinational study of the European Network for the Study of Adrenal Tumors (ENS@T). Modern Pathology28807\u2013821. (https:\/\/doi.org\/10.1038\/modpathol.2015.41)"},{"key":"bib35","series-title":"Endocrine Pathology","first-page":"396","article-title":"A subset of large cell neuroendocrine carcinomas in the gastroenteropancreatic tract may evolve from pre-existing well-differentiated neuroendocrine tumors","volume":"32","author":"Pelosi","year":"2021","unstructured":"Pelosi GBianchi FDama EMetovic JBarella MSonzogni AAlbini APapotti MGong Y & Vijayvergia N2021A subset of large cell neuroendocrine carcinomas in the gastroenteropancreatic tract may evolve from pre-existing well-differentiated neuroendocrine tumors. Endocrine Pathology32396\u2013407. (https:\/\/doi.org\/10.1007\/s12022-020-09659-6)"},{"key":"bib36","series-title":"British Journal of Cancer","first-page":"429","article-title":"Immunohistochemical markers of the hypoxic response can identify malignancy in phaeochromocytomas and paragangliomas and optimize the detection of tumours with VHL germline mutations","volume":"108","author":"Pinato","year":"2013","unstructured":"Pinato DJRamachandran RToussi STVergine MNgo NSharma RLloyd TMeeran KPalazzo FMartin N, et al.2013Immunohistochemical markers of the hypoxic response can identify malignancy in phaeochromocytomas and paragangliomas and optimize the detection of tumours with VHL germline mutations. British Journal of Cancer108429\u2013437. (https:\/\/doi.org\/10.1038\/bjc.2012.538)"},{"key":"bib37","series-title":"Frontiers in Oncology","first-page":"780716","article-title":"Biomarker landscape in neuroendocrine tumors with high-grade features: current knowledge and future perspective","volume":"12","author":"Prisciandaro","year":"2022","unstructured":"Prisciandaro MAntista MRaimondi ACorti FMorano FCentonze GSabella GMangogna ARandon GPagani F, et al., 2022Biomarker landscape in neuroendocrine tumors with high-grade features: current knowledge and future perspective. Frontiers in Oncology12780716."},{"key":"bib38","series-title":"Histopathology","first-page":"454","article-title":"Extrapulmonary small cell carcinoma: a clinicopathological study with identification of potential diagnostic mimics","volume":"61","author":"Quinn","year":"2012","unstructured":"Quinn AMBlackhall FWilson GDanson SClamp AAshcroft LBrierley J & Hasleton P2012Extrapulmonary small cell carcinoma: a clinicopathological study with identification of potential diagnostic mimics. Histopathology61454\u2013464. (https:\/\/doi.org\/10.1111\/j.1365-2559.2012.04247.x)"},{"key":"bib39","series-title":"Modern Pathology","first-page":"1770","article-title":"A common classification framework for neuroendocrine neoplasms: an International Agency for Research on Cancer (IARC) and World Health Organization (WHO) expert consensus proposal","volume":"31","author":"Rindi","year":"2018","unstructured":"Rindi GKlimstra DSAbedi-Ardekani BAsa SLBosman FTBrambilla EBusam KJde Krijger RRDietel MEl-Naggar AK, et al.2018A common classification framework for neuroendocrine neoplasms: an International Agency for Research on Cancer (IARC) and World Health Organization (WHO) expert consensus proposal. Modern Pathology311770\u20131786. )https:\/\/doi.org\/10.1038\/s41379-018-0110-y)"},{"key":"bib40","series-title":"Endocrine Pathology","first-page":"115","article-title":"Overview of the 2022 WHO classification of neuroendocrine neoplasms","volume":"33","author":"Rindi","year":"2022","unstructured":"Rindi GMete OUccella SBasturk OLa Rosa SBrosens LAAEzzat Sde Herder WWKlimstra DSPapotti M, et al.2022Overview of the 2022 WHO classification of neuroendocrine neoplasms. Endocrine Pathology33115\u2013154. (https:\/\/doi.org\/10.1007\/s12022-022-09708-2)"},{"key":"bib41","series-title":"Lung Cancer","first-page":"149","article-title":"Lung carcinoids with high proliferative activity: further support for the identification of a new tumor category in the classification of lung neuroendocrine neoplasms","volume":"148","author":"Rubino","year":"2020","unstructured":"Rubino MScoazec JYPisa EFaron MSpaggiari LHadoux JSpada FPlanchard DCella CALeboulleux S, et al.2020Lung carcinoids with high proliferative activity: further support for the identification of a new tumor category in the classification of lung neuroendocrine neoplasms. Lung Cancer148149\u2013158. (https:\/\/doi.org\/10.1016\/j.lungcan.2020.08.001)"},{"key":"bib42","series-title":"Journal of Clinical Endocrinology and Metabolism","first-page":"1163","article-title":"Multiple endocrine tumors associated with germline MAX mutations: multiple endocrine neoplasia type 5?","volume":"106","author":"Seabrook","year":"2021","unstructured":"Seabrook AJHarris JEVelosa SBKim EMcInerney-Leo AMDwight THockings JIHockings NGKirk JLeo PJ, et al.2021Multiple endocrine tumors associated with germline MAX mutations: multiple endocrine neoplasia type 5?Journal of Clinical Endocrinology and Metabolism1061163\u20131182. (https:\/\/doi.org\/10.1210\/clinem\/dgaa957)"},{"key":"bib43","series-title":"Cancers (Basel)","first-page":"225","article-title":"The value of histological algorithms to predict the malignancy potential of pheochromocytomas and abdominal paragangliomas-a meta-analysis and systematic review of the literature","volume":"11","author":"Stenman","year":"2019","unstructured":"Stenman AZedenius J & Juhlin CC2019The value of histological algorithms to predict the malignancy potential of pheochromocytomas and abdominal paragangliomas-a meta-analysis and systematic review of the literature. Cancers (Basel)11225. (https:\/\/doi.org\/10.3390\/cancers11020225)"},{"key":"bib44","series-title":"Pathology International","first-page":"411","article-title":"High-grade transformation of pancreatic neuroendocrine tumor associated with TP53 mutations: a diagnostic pitfall mimicking neuroendocrine carcinoma","volume":"72","author":"Tanaka","year":"2022","unstructured":"Tanaka MShinozaki-Ushiku AKunita AYasunaga YAkamatsu NHasegawa K & Ushiku T2022High-grade transformation of pancreatic neuroendocrine tumor associated with TP53 mutations: a diagnostic pitfall mimicking neuroendocrine carcinoma. Pathology International72411\u2013418. (https:\/\/doi.org\/10.1111\/pin.13252)"},{"key":"bib45","series-title":"Modern Pathology","first-page":"1571","article-title":"INSM1 expression in a subset of thoracic malignancies and small round cell tumors: rare potential pitfalls for small cell carcinoma","volume":"33","author":"Tsai","year":"2020","unstructured":"Tsai HKHornick JL & Vivero M2020INSM1 expression in a subset of thoracic malignancies and small round cell tumors: rare potential pitfalls for small cell carcinoma. Modern Pathology331571\u20131580. (https:\/\/doi.org\/10.1038\/s41379-020-0517-0)"},{"key":"bib46","series-title":"Endocrine Pathology","first-page":"351","article-title":"Calcitonin-producing neuroendocrine neoplasms of the pancreas: clinicopathological study of 25 cases and review of the literature","volume":"28","author":"Uccella","year":"2017","unstructured":"Uccella SBlank AMaragliano RSessa FPerren A & La Rosa S2017Calcitonin-producing neuroendocrine neoplasms of the pancreas: clinicopathological study of 25 cases and review of the literature. Endocrine Pathology28351\u2013361. (https:\/\/doi.org\/10.1007\/s12022-017-9505-4)"},{"key":"bib48","series-title":"Endocrine Pathology","first-page":"150","article-title":"Immunohistochemical biomarkers of gastrointestinal, pancreatic, pulmonary, and thymic neuroendocrine neoplasms","volume":"29","author":"Uccella","year":"2018","unstructured":"Uccella SLa Rosa SVolante M & Papotti M2018Immunohistochemical biomarkers of gastrointestinal, pancreatic, pulmonary, and thymic neuroendocrine neoplasms. Endocrine Pathology29150\u2013168. (https:\/\/doi.org\/10.1007\/s12022-018-9522-y)"},{"key":"bib47","series-title":"Endocrine Pathology","first-page":"192","article-title":"Genomics of high-grade neuroendocrine neoplasms: well-differentiated neuroendocrine tumor with high-grade features (G3 NET) and neuroendocrine carcinomas (NEC) of various anatomic sites","volume":"32","author":"Uccella","year":"2021","unstructured":"Uccella SLa Rosa SMetovic JMarchiori DScoazec JYVolante MMete O & Papotti M2021Genomics of high-grade neuroendocrine neoplasms: well-differentiated neuroendocrine tumor with high-grade features (G3 NET) and neuroendocrine carcinomas (NEC) of various anatomic sites. Endocrine Pathology32192\u2013210. (https:\/\/doi.org\/10.1007\/s12022-020-09660-z)"},{"key":"bib49","series-title":"Endocrine-Related Cancer","first-page":"e230021","article-title":"The World Health Organization classifications of pituitary neuroendocrine tumours: a clinico-pathological appraisal","volume":"30","author":"Villa","year":"2023","unstructured":"Villa CBaussart BAssi\u00e9 GRaverot G & Roncaroli F2023The World Health Organization classifications of pituitary neuroendocrine tumours: a clinico-pathological appraisal. Endocrine-Related Cancer30e230021. (https:\/\/doi.org\/10.1530\/ERC-23-0021)"},{"key":"bib50","series-title":"Endocrine Pathology","first-page":"154","article-title":"Molecular pathology of well-differentiated pulmonary and thymic neuroendocrine tumors: what do pathologists need to know?","volume":"32","author":"Volante","year":"2021","unstructured":"Volante MMete OPelosi GRoden ACSpeel EJM & Uccella S2021Molecular pathology of well-differentiated pulmonary and thymic neuroendocrine tumors: what do pathologists need to know?Endocrine Pathology32154\u2013168. (https:\/\/doi.org\/10.1007\/s12022-021-09668-z)"},{"key":"bib51","series-title":"Journal of Neuroendocrinology","first-page":"e13260","article-title":"Baseline tumor growth rate highlights the heterogeneity of well differentiated gastroenteropancreatic neuroendocrine tumors and predicts for increases in Ki67 index over time","volume":"35","author":"Wang","year":"2023","unstructured":"Wang SJWhitman JPaciorek ALe BKNakakura EKBehr SCJoseph NZhang LHope TA & Bergsland EK2023Baseline tumor growth rate highlights the heterogeneity of well differentiated gastroenteropancreatic neuroendocrine tumors and predicts for increases in Ki67 index over time. Journal of Neuroendocrinology35e13260. (https:\/\/doi.org\/10.1111\/jne.13260)"},{"key":"bib52","series-title":"Journal of Clinical Oncology","first-page":"96","article-title":"International medullary thyroid carcinoma grading system: a validated grading system for medullary thyroid carcinoma","volume":"40","author":"Xu","year":"2022","unstructured":"Xu BFuchs TLAhmadi SAlghamdi MAlzumaili BBani MABaudin EChou ADe Leo AFagin JA, et al.2022International medullary thyroid carcinoma grading system: a validated grading system for medullary thyroid carcinoma. Journal of Clinical Oncology4096\u2013104. (https:\/\/doi.org\/10.1200\/JCO.21.01329)"},{"key":"bib53","series-title":"American Journal of Surgical Pathology","first-page":"173","article-title":"Small cell and large cell neuroendocrine carcinomas of the pancreas are genetically similar and distinct from well-differentiated pancreatic neuroendocrine tumors","volume":"36","author":"Yachida","year":"2012","unstructured":"Yachida SVakiani EWhite CMZhong YSaunders TMorgan Rde Wilde RFMaitra AHicks JDemarzo AM, et al.2012Small cell and large cell neuroendocrine carcinomas of the pancreas are genetically similar and distinct from well-differentiated pancreatic neuroendocrine tumors. American Journal of Surgical Pathology36173\u2013184. (https:\/\/doi.org\/10.1097\/PAS.0b013e3182417d36)"},{"key":"bib54","series-title":"Cancers (Basel)","first-page":"103","article-title":"Merkel cell carcinoma-update on diagnosis, management and future perspectives","volume":"15","author":"Zaggana","year":"2022","unstructured":"Zaggana EKonstantinou MPKrasagakis GHde Bree EKalpakis KMavroudis D & Krasagakis K2022Merkel cell carcinoma-update on diagnosis, management and future perspectives. Cancers (Basel)15103. (https:\/\/doi.org\/10.3390\/cancers15010103)"},{"key":"bib55","series-title":"Endocrine","first-page":"61","article-title":"Insulinoma-associated protein 1(INSM1) is a superior marker for the diagnosis of gastroenteropancreatic neuroendocrine neoplasms: a meta-analysis","volume":"74","author":"Zhang","year":"2021","unstructured":"Zhang QHuang JHe YCao R & Shu J2021Insulinoma-associated protein 1(INSM1) is a superior marker for the diagnosis of gastroenteropancreatic neuroendocrine neoplasms: a meta-analysis. 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