Skip to main content
SpringerLink
Log in

Carcinoid Syndrome

  • Living reference work entry
  • First Online:
Encyclopedia of Pathology

Part of the book series: Encyclopedia of Pathology ((EP))

  • 34 Accesses

Synonyms

Carcinoid disease

Definition

Carcinoid syndrome is a paraneoplastic syndrome comprising different signs and symptoms, mainly affecting the gastrointestinal and vascular systems, in a patient affected by neuroendocrine tumor (NET). Generally major symptoms of the syndrome include flushing, diarrhea, and wheezing, but clinical presentation is heterogeneous. Carcinoid syndrome is more common in well-differentiated NET arising from midgut, mainly in patients with high disease burden and liver metastases and with high release of serotonin, the major bioactive tumor-released product, into the systemic circulation (Rubin de Celis Ferrari et al. 2018).

Clinical Features

  • Incidence

    Carcinoid syndrome is the most frequent of the NET-related hormonal syndromes. Approximately 30–40% of patients with NET present with features of the carcinoid syndrome. According to data from the Surveillance, Epidemiology, and End Results (SEER) database, incidence of carcinoid syndrome is increasing,...

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Log in via an institution

Institutional subscriptions

References and Further Reading

  • Davar, J., Connolly, H. M., Caplin, M. E., Pavel, M., Zacks, J., Bhattacharyya, S., Cuthbertson, D. J., Dobson, R., Grozinsky-Glasberg, S., Steeds, R. P., Dreyfus, G., Pellikka, P. A., & Toumpanakis, C. (2017). Diagnosing and managing carcinoid heart disease in patients with neuroendocrine tumors: An expert statement. Journal of the American College of Cardiology, 69(10), 1288–1304.

    Article  Google Scholar 

  • Halperin, D. M., Shen, C., Dasari, A., Xu, Y., Chu, Y., Zhou, S., Shih, Y. T., & Yao, J. C. (2017). The frequency of carcinoid syndrome at neuroendocrine tumor diagnosis: A large population-based study using SEER-Medicare data. The Lancet Oncology, 18, 525–534.

    Article  Google Scholar 

  • Hofland, J., Herrera Martinez, A. D., Zandee, W. T., & de Herder, W. W. (2019). Management of carcinoid syndrome: A systematic review and meta-analysis. Endocrine-Related Cancer, 26, R145–R156.

    Article  Google Scholar 

  • Ito, T., Lingaku, L., & Jensen, R. T. (2018). Carcinoid-syndrome: Recent advances, current status and controversies. Current Opinion in Endocrinology, Diabetes and Obesity, 25(1), 22–35.

    Article  Google Scholar 

  • Loughrey, P. B., Zhang, D., & Heaney, A. P. (2018). New treatments for the carcinoid syndrome. Endocrinology and Metabolism Clinics of North America, 47(3), 557–576.

    Article  Google Scholar 

  • Rubin de Celis Ferrari, A. C., Glasberg, J., & Riechelmann, R. P. (2018). Carcinoid syndrome: Update on the pathophysiology and treatment. Clinics, 73(Suppl 1), e490s.

    PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Dipartimento di Medicina Clinica e Chirurgia, University “Federico II” of Naples, Naples, Italy

    Annamaria Colao, Claudia Pivonello, Roberta Modica & Filomena Bottiglieri

Authors
  1. Annamaria Colao
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Claudia Pivonello
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Roberta Modica
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Filomena Bottiglieri
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Annamaria Colao .

Editor information

Editors and Affiliations

  1. NIJMEGEN, The Netherlands

    J.H.J.M. van Krieken

Rights and permissions

Reprints and permissions

Copyright information

© 2020 Springer Nature Switzerland AG

About this entry

Check for updates. Verify currency and authenticity via CrossMark

Cite this entry

Colao, A., Pivonello, C., Modica, R., Bottiglieri, F. (2020). Carcinoid Syndrome. In: van Krieken, J. (eds) Encyclopedia of Pathology. Encyclopedia of Pathology. Springer, Cham. https://doi.org/10.1007/978-3-319-28845-1_5102-1

Download citation

  • DOI: https://doi.org/10.1007/978-3-319-28845-1_5102-1

  • Received:

  • Accepted:

  • Published:

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-28845-1

  • Online ISBN: 978-3-319-28845-1

  • eBook Packages: Springer Reference MedicineReference Module Medicine

Publish with us

Policies and ethics

Search

Navigation