Synonyms
Definition
Atypical carcinoid syndrome is a clinical variant of the typical carcinoid syndrome, a paraneoplastic syndrome comprising different signs and symptoms, mainly affecting the vascular and bronchial systems, in a patient affected by neuroendocrine tumor (NET). Generally major symptoms of the syndrome include flushing and wheezing, unlike the typical where diarrhea is mostly reported. Atypical carcinoid syndrome is more common in well-differentiated NET arising from the foregut, including the lung, stomach, and duodenum. Histamine is considered the major bioactive tumor-released product, and symptoms are related to its overproduction (Kaltsas et al. 2017).
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. In only 5% of cases, carcinoid syndrome is defined as atypical (Tomassetti et al. 2001). No...
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References and Further Reading
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.
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.
Kaltsas, G., Caplin, M., Davies, P., Ferone, D., Garcia-Carbonero, R., Grozinsky-Glasberg, S., Hörsch, D., Tiensuu Janson, E., Kianmanesh, R., Kos-Kudla, B., Pavel, M., Rinke, A., Falconi, M., & de Herder, W. W. (2017). ENETS consensus guidelines for the standards of care in neuroendocrine tumors: Pre- and perioperative therapy in patients with neuroendocrine tumors. Neuroendocrinology, 105(3), 245–254.
Rastogi, V., Singh, D., Mazza, J. J., Parajuli, D., & Yale, S. H. (2017). Flushing disorders associated with gastrointestinal symptoms: Part 1, neuroendocrine tumors, mast cell disorders and hyperbasophila. Clinical Medicine & Research, 16(1–2), 16–28.
Tomassetti, P., Migliori, M., Lalli, S., Campana, D., Tomassetti, V., & Corinaldesi, R. (2001). Epidemiology, clinical features and diagnosis of gastroenteropancreatic endocrine tumours. Annals of Oncology, 12(Suppl 2), S95–S99.
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Colao, A., Pivonello, C., Modica, R., Bottiglieri, F. (2020). Atypical 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_5101-1
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DOI: https://doi.org/10.1007/978-3-319-28845-1_5101-1
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