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Neuroendocrine Neoplasms (NENs)

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Practical Medical Oncology Textbook

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Abstract

Neuroendocrine neoplasms (NENs) represent a rare and heterogeneous group of malignancies which can develop in many different sites of our body. They originate from the cells of the diffuse neuroendocrine system. Gastroenteropancreatic NENs were classified in four categories, including NETs G1 (WD with <3% Ki-67), NETs G2 (WD with 3–20% Ki-67), NETs G3 (WD with >20% Ki-67), and NECs (PD with >20% Ki-67) in accordance with the 2019 WHO classification (IARC WHO Classification of the digestive system tumors, on 11th July 2019). Lung NENs, in accordance with the latest WHO classification, 2015 edition, are distinguished in small cell lung cancer (SCLC), large cell neuroendocrine carcinoma (LCNEC), atypical carcinoid (AC), and typical carcinoid (TC).

From a clinical perspective, it is critical to distinguish GEP NENs in functioning and nonfunctioning. The former regards the presence of a clinical syndrome related to the production of one or more substances or hormones by the tumor, whereas the latter indicates the absence of a clinical syndrome related to the tumor although the patient can be symptomatic due to mass-effect symptoms related to the tumor and/or the tumor can secrete some substances without any clinical implication.

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Authors and Affiliations

  1. Division of Gastrointestinal Medical Oncology and Neuroendocrine Tumors, European Institute of Oncology, IEO, IRCCS, Milan, Italy

    Nicola Fazio & Francesca Spada

  2. Gastrointestinal and Hepato-Pancreatic Surgery and Liver Transplantation Unit, Fondazione IRCCS Istituto Nazionale Tumori (INT, National Cancer Institute) and Università degli Studi di Milano, Milan, Italy

    Roberta Elisa Rossi

  3. Nuclear Medicine Service - PET Unit, Policlinico S. Orsola-Malpighi, Bologna University, Bologna, Italy

    Valentina Ambrosini

  4. Department of Biomedicine, Neuroscience and Advanced Diagnostics, University of Palermo, Palermo, Italy

    Lorena Incorvaia

  5. Department of Surgical, Oncological and Oral Science, University of Palermo, Palermo, Italy

    Massimiliano Cani & Giuseppe Badalamenti

  6. Department of Oncology, University of Torino, AOU S. Luigi Gonzaga, Orbassano, Italy

    Francesco Passiglia

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  1. Nicola Fazio
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  8. Giuseppe Badalamenti
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Correspondence to Nicola Fazio .

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Editors and Affiliations

  1. Department of Surgical, Oncological and Oral Sciences, University of Palermo, Palermo, Italy

    Antonio Russo

  2. Oncology Department, University of Antwerp, Edegem, Belgium

    Marc Peeters

  3. Department of Biomedicine, Neuroscience and Advanced Diagnostics, University of Palermo, Palermo, Italy

    Lorena Incorvaia

  4. Center for Thoracic Oncology, Tisch Cancer Institute, Mount Sinai System & Icahn School of Medicine, Mount Sinai, NY, USA

    Christian Rolfo

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Fazio, N. et al. (2021). Neuroendocrine Neoplasms (NENs). In: Russo, A., Peeters, M., Incorvaia, L., Rolfo, C. (eds) Practical Medical Oncology Textbook. UNIPA Springer Series. Springer, Cham. https://doi.org/10.1007/978-3-030-56051-5_60

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