Abstract

Carcinoid syndrome (CS) is a paraneoplastic syndrome caused by the release of serotonin and other substances from well-differentiated neuroendocrine tumors (NETs). The hallmark symptoms of carcinoid syndrome are flushing and diarrhea; atypical signs and symptoms can include wheezing, abdominal pain, valvular heart disease, telangiectasias, pellagra, and the complications of mesenteric fibrosis, including ureteral obstruction, bowel obstruction, and bowel ischemia. These symptoms are mediated by the release of serotonin (5-HT), histamine, kallikrein, prostaglandins, and tachykinins. The diagnosis of CS requires these symptoms and corresponding elevations in lab tests. Treatment options include surgery and medical management with somatostatin analogs.

Highlights

  • BackgroundCarcinoid syndrome (CS) is a paraneoplastic syndrome caused by the release of serotonin and other substances from well-differentiated neuroendocrine tumors (NETs) [1]

  • A carcinoid crisis is a potentially life-threatening complication of carcinoid syndrome caused by the sudden release of 5-HT and other vasoactive peptides, such as histamine, kallikreins, or catecholamines, which are precipitated by tumor manipulation during surgery, percutaneous needle biopsy, or even anesthesia [1,16]

  • Blood/ urine serotonin levels are not recommended as a standard diagnostic test as the end product of the serotonin metabolism is 5-hydroxy indole acetic acid (5-HIAA) [30]

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Summary

Introduction

Carcinoid syndrome (CS) is a paraneoplastic syndrome caused by the release of serotonin and other substances from well-differentiated neuroendocrine tumors (NETs) [1]. A carcinoid crisis is a potentially life-threatening complication of carcinoid syndrome caused by the sudden release of 5-HT and other vasoactive peptides, such as histamine, kallikreins, or catecholamines, which are precipitated by tumor manipulation during surgery, percutaneous needle biopsy, or even anesthesia [1,16]. It manifests as hypotension or hypertension, diarrhea, bronchoconstriction, flushing, and acidosis [17]. The bad prognostic factors are carcinoid heart disease and high levels of tumor markers, liver metastasis, the involvement of the thymus, overexpression of the proliferation of Ki67, and mutation of the p53 gene [5]

Conclusions
Disclosures
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11. Pitche PT
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