Carcinoid Tumors : Mnemonic

Origin: Neuroendocrine cells

Location: Lungs, Bronchi, Trachea, Gastrointestinal tract

Synthesize: Biogenic amines (mainly serotonin which is metabolied to 5-HIAA)

5 Manifestations

Mnemonic: 5-HIAAS

1. Heart disease (2/3 patients)

• Right sided valvular heart lesions (due to factors secreted into hepatic vein by liver metastases)
  • Tricuspid regurgitation
  • Tricuspid stenosis
  • Pulmonary stenosis (less common)
• Left sided heart disease (infrequent): 10%
• Hypotension

2. Intestinal manifestations

• Gastrointestinal bleeding
• Obstruction
• Watery diarrhea unresponsive to fasting

3. Airway obstruction

• Localized wheezing +/- hemoptysis

4. Asthma

• Significant diffuse wheezing

5. Skin manifestations

• Intense cutaneous flushing
• Necrolytic erythema

Alternative mnemonic: CARCinoid

1. Cutaneous flushing
2. Asthmatic wheezing
3. Right sided valvular heart lesions
4. Cramping and diarrhea

Rule of 1/3

• 1/3 of carcinoid tumors metastasize.
• 1/3 of carcinoid tumors present with 2nd malignancy.
• 1/3 of carcinoid tumors are multiple.

Surgery for appendiceal carcinoid tumor

1. <2 cm: Simple appendectomy
2. >2 cm or mesoappendix invasion: Hemicolectomy

Medical therapy

1. Somatostatin analogue: Octreotide
2. Tryptophan hydroxylase inhibitor: Telotristat