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Carcinoid: Presentation and Diagnosis, Surgical Management

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Endocrine Surgery

Part of the book series: Springer Specialist Surgery Series ((SPECIALIST))

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The name “carcinoid” was first used by Oberndorfer in 1907 to describe uncommon ileal tumors with benign behavior in contrast to common bowel carcinomas. The term was subsequently used as an overall name for neuroendocrine cell-derived tumors. Based on their embryological origin these tumors have commonly been classified into foregut, midgut, and hindgut carcinoids. In recent classifications the carcinoids have been named neuroendocrine tumors of the respective organs, and classified into the categories: well-differentiated neuroendocrine tumors, well-differentiated endocrine carcinoma, poorly differentiated endocrine carcinomas, and mixed exocrine–endocrine tumors [1]. In this chapter we will cover the clinical presentation, diagnosis and surgical management of carcinoids, and for clarity have used a common older classification. Carcinoids are rare with an incidence of 1–2/100,000 population/year. About 70% occur in the gastrointestinal tract [2]. They typically stain positively for chromogranin A and/or synaptophysin. The majority are differentiated (with low mitotic activity and low Ki67 proliferation index, often <2%), while others are intermediate or poorly differentiated, with an increased mitotic rate and higher proliferation (Ki67 index 10–40%) [1]. Most carcinoids are clinically nonfunctional, but some have dominant secretion (serotonin, histamine, gastrin, somatostatin), sometimes causing severe symptoms. The most common is the carcinoid syndrome, associated with the “classical” midgut jejuno-ileal carcinoid. Rare foregut carcinoids can have ectopic adrenocorticotropic hormone (ACTH) or corticotropin-releasing factor (CRF) secretion, causing ectopic Cushing’s syndrome [1]. The well-differentiated tumors often have an extended clinical course and therefore have specific surgical requirements based on tumor type and localization.

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Authors and Affiliations

  1. University Hospital, Uppsala, Sweden

    Göran Åkerström (MD)

  2. Department of Surgery, Endocrine Surgical Unit, University Hospital, Uppsala, Sweden

    Per Hellman (MD, PhD) & Peter Stålberg (MD, PhD)

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  1. Göran Åkerström
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  2. Per Hellman
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Editors and Affiliations

  1. St Thomas Hospital, Lambeth Palace Road, London SE1 7EH, United Kingdom

    Johnathan Hubbard

  2. Coll Physicians & Surgeons Columbia, Fort Washington Avenue 161, New York, 10032, U.S.A.

    William B. Inabnet

  3. Queen Mary Hospital, University of Hong Kong Medical Centre, Pokfulam, Hong Kong, China, People's Republic

    Chung-Yau Lo

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Åkerström, G., Hellman, P., Stålberg, P. (2009). Carcinoid: Presentation and Diagnosis, Surgical Management. In: Hubbard, J., Inabnet, W., Lo, CY. (eds) Endocrine Surgery. Springer Specialist Surgery Series. Springer, London. https://doi.org/10.1007/978-1-84628-881-4_44

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  • DOI: https://doi.org/10.1007/978-1-84628-881-4_44

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