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      Neuroendocrine neoplasm update: toward universal nomenclature

      1 , 2 , 3 , 1 , 3

      Endocrine-Related Cancer

      Bioscientifica

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          Abstract

          Neuroendocrine neoplasia is described in almost every tissue, either in the pure endocrine organs, the nerve structures or in the so-called diffuse neuroendocrine system. The current nomenclature contains time-honored, widely accepted definitions; however, it is different according to anatomical sites. Diverse definitions may generate confusion and non-standard patient management. The International Agency for Research on Cancer – World Health Organization (IARC-WHO) proposed a framework for universal classification of neuroendocrine neoplasia. Evidence indicates that neuroendocrine cancer is composed by cells with a distinctive phenotype characterized by the expression of general and specific neuroendocrine markers. The neuroendocrine phenotype is indicated as descriptor of a unique cancer category, now recommended for all organs as neuroendocrine neoplasm. Evidence indicates that neuroendocrine neoplasia may be well or poorly differentiated, with diverse incidence and prevalence in different organs. It is proposed that the well-differentiated neoplasm is universally defined as neuroendocrine tumor (NET) and the poorly differentiated as neoplasm neuroendocrine carcinoma (NEC). Evidence indicates that a cancer grading tool based on a proliferation measure by mitotic count, Ki67 % and/or necrosis assessment is useful to predict NET patient behavior. It is proposed to utilize this tool for grading NET universally, with site-specific cut-offs to be defined. It is also acknowledged that significant biological site-specific differences exist. It is recommended that current pathology reports contain this classification together with the current traditional classifiers. This IARC-WHO common classification framework for neuroendocrine neoplasm aims at uniformizing nomenclature toward different organs and at fostering the definition of a similar site-specific gene signature.

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          Author and article information

          Journal
          Endocrine-Related Cancer
          Bioscientifica
          1351-0088
          1479-6821
          June 2020
          June 2020
          June 2020
          June 2020
          : 27
          : 6
          : R211-R218
          Affiliations
          [1 ]1Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, Italy
          [2 ]2Università Cattolica del Sacro Cuore, Rome, Italy
          [3 ]3ENETS Center of Excellence, Neuroendocrine Tumour (NET) Center, Rome, Italy
          Article
          10.1530/ERC-20-0036
          © 2020

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