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Updated: May 14 2017

Carcinoid Syndrome

Introduction
  • Tumor of neuroendocrine origin
  • Location
    • determines whether or not carcinoid syndrome is present
      • no carcinoid syndrome if the GI mass has not metastasized to the liver
        • liver metabolizes 5-HT arriving in the portal circulation
      • carcinoid syndrome is observed if tumor exists outside GI system
  • Most common site of carcinoid tumor is appendix but most common site of carcinoid tumor causing carcinoid syndrome is the small bowel
  • Patients at risk for developing niacin deficiency 
    • all tryptophan is consumed in making excess 5-HT
Presentation
  • Symptoms
    • diarrhea
      • ↑ bowel motility
    • flushing
  • Phyical exam
    • right-sided heart murmurs
      • tricuspid insufficiency, pulmonary stenosis
    • wheezing
      • ↑ bronchospasm
Evaluation
  • Biopsy
    • bright yellow tumor
  • CT scan of liver
    • detect masses
  • Urinalysis
    • 5-HT degraded to 5-HIAA and may be detected in the urine
Treatment
  • Surgery
    • first line is excision
  • Medical (if surgery not possible)
    • octreotide 
      • somatostatin analogue
      • controls flushing and diarrhea
Question
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