Carcinoid tumors are slow-growing, usually asymptomatic neuroendocrine tumors of enterochromaffin cell origin. Functional neuroendocrine tumors secrete many different mediators such as histamine, 5-hydroxytryptamine (serotonin), and 5-hydroxytryptophan. Carcinoid syndrome is characterized by episodic flushing, diarrhea, wheezing, and right heart valve disease due to these mediators. Carcinoid crisis is a very serious complication of carcinoid syndrome and defined as the severe combination of carcinoid syndrome symptoms. The risk of carcinoid crisis varies between 3.4% and 24%. There are many factors triggering the carcinoid crisis. The most common cause is anesthesia and surgery.
There are no well-defined predicting risk factors for the development of carcinoid crisis. However there are some risk factors such as presence of carcinoid syndrome, elevated 5-HIAA, high tumor burden, metastatic disease, carcinoid heart disease, liver metastases, duration of anesthesia, and advanced patient age.
The aim of the treatment of carcinoid crisis is to block the secretion and effect of mediators released by the tumor and control the symptoms. The cornerstone of this treatment is somatostatin analogs. Other drugs used in the treatment of carcinoid crisis are methylene blue, ketanserin, and H1 and H2 receptor antagonists.
Carcinoid syndrome Carcinoid crisis Somatostatin analog Octreotide Lanreotide Ketanserin Histamine Methylene blue
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