Carcinoid syndrome classification

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Anum Gull M.B.B.S.[2]Parminder Dhingra, M.D. [3]

Overview

Gastroenteropancreatic neuroendocrine tumors are classified based upon their origin from the embryonic divisions of the alimentary tract into foregut (bronchial, stomach), midgut (small intestine, appendix, cecum) and hindgut (distal colon, rectum, genitourinary) tumours.

Classification

• Gastroenteropancreatic neuroendocrine tumors are classified based upon their origin from the embryonic divisions of the alimentary tract:

1. Foregut (bronchial, stomach)
2. Midgut (small intestine, appendix, cecum)
3. Hindgut (distal colon, rectum, genitourinary)

• GET-NETs produce a number of secretory products, resulting in a wide range of clinical symptoms.
• Midgut gastrointestinal tract NETs (GINETs) produce serotonin and other vasoactive substances that give rise to the typical carcinoid syndrome.
• Lung neuroendocrine tumors produce lesser quantities of serotonin.
• carcinoid syndrome is caused less commonly by lung neuroendocrine tumor and most often with tumors of large size (>5 cm).

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Gastroenteropancreatic neuroendocrine tumors

	Foregut	Midgut	Hindgut
Location	Stomach Duodenum Bronchus Thymus	Jejunum Ileum Appendix Ascending colon	Transverse colon Descending colon Sigmoid colon Rectum Genitourinary
Hormones produced	5-hydroxytryptophan Histamine Multiple polypeptides	Serotonin Prostaglandins Polypeptides	Variable
Possibility of carcinoid syndrome	Rare, and atypical when it happens	Classic	Rare