Carcinoid syndrome causes

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

Overview

Common causes of carcinoid syndrome include genetic disorders (multiple endocrine neoplasia type 1 and neurofibromatosis type 1) and genetic mutations (gains involving chromosomes 5, 14, 17, and 19 and losses involving chromosomes 11 and 18).

Causes

• Approximately 30-40% of patients with well-differentiated neuroendocrine tumors present with carcinoid syndrome.
• Carcinoid syndrome is predominantly associated with neuroendocrine tumors (NETs) that arise from the midgut in the setting of extensive liver metastases
• Carcinoid syndrome may be present in patients with bronchial carcinoids.
• other common causes of carcinoid syndrome includes are mostly genetic:
• Genetic disorders

Genetic Disorder	Tumor Location
Multiple endocrine neoplasia type 1	Carcinoids associated with multiple endocrine neoplasia type 1 appear to be of foregut origin
Neurofibromatosis type 1	Carcinoids in patients with neurofibromatosis type 1 appear to arise primarily in the periampullary region

• Genetic mutations

Type of Mutation	Chromosomes
Gains	Chromosome 5 Chromosome 14 Chromosome 17 Chromosome 19
Losses	Chromosome 11 Chromosome 18