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 on 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

• Gastroentero-pancreatic neuroendocrine tumors are classified based on 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)

• Gasroenteropancreatic- neuroendocrine tumor produce a number of secretory products, resulting in a wide range of clinical symptoms.
• Midgut gastrointestinal tract neuroendocrine tumors produce serotonin and other vasoactive substances that causes the manifestations of typical carcinoid syndrome.
• Lung neuroendocrine tumors produce less quantities of serotonin.
• Carcinoid syndrome is caused less commonly by lung neuroendocrine tumor and most often by 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 occurs	Classic	Rare