Updated: 8/29/2019

Carcinoid Tumor

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Introduction
  • Tumor of neuroendocrine origin
    • most common small bowel tumor
  • Location
    • most common sites are the rectum and stomach
      • these foregut and hindgut masses rarely metastasize
    • most common site of metastatic primary mass is terminal ileum
      • appendix is common tumor site but too small to metastasize
    • location determines whether or not carcinoid syndrome is present
      • no carcinoid syndrome if the 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
  • Pathophysiology
    • often produce 5-HT ectopically
      • can lead to carcinoid syndrome 
Evaluation
  • Biopsy
    • dense core bodies seen on EM
      • bodies are secretory granules
    • bright yellow tumor
  • Urinalysis
    • 5-HT degraded to 5-HIAA and may be detected in the urine  
Treatment
  • Pharmacologic
    • hormone therapy
      • somatostatin analogue to prevent extra hormones from being produced
      • octreotide or lanreotide can help lessen flushing and diarrhea
    • radiation and chemotherapy
  • Surgical
    • surgical resection
      • can be done endoscopically, with local excision or with cryosurgery

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(M1.GI.13.55) A 53-year-old female visits her physician with watery diarrhea and episodic flushing. The patient reports that she is often short of breath, and a pulmonary exam reveals bilateral wheezing. A CT scan shows a mass in the terminal ileum. 24-hour urine collection shows abnormally elevated 5-hydroxyindoleacetic acid (HIAA) levels. Ultrasound demonstrates a tricuspid valve with signs of fibrosis with a normal mitral valve. A metastatic disease to which organ is most commonly associated with the patient's syndrome?

QID: 101112
1

Lung

18%

(14/76)

2

Kidney

4%

(3/76)

3

Brain

1%

(1/76)

4

Liver

62%

(47/76)

5

Pancreas

7%

(5/76)

M 2 E

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