History and exam
Key diagnostic factors
common
diarrhea
Common symptom (75% of cases), variable in severity, often present for many years prior to diagnosis.[15] Mediated by serotonin.
flushing
Often a long-standing symptom, occurring in 85% of cases, associated initially with stress and precipitants such as tyramine-containing foods and alcohol. Usually sudden onset with a short duration of a few minutes, affecting face and upper trunk.
Other diagnostic factors
common
palpitations
Usually episodic and may be associated with tachycardia.
abdominal cramps
Common (40%), marked by acute onset and spontaneous settling.[8] Often associated with diarrhea.
telangiectasia
Occurs in 25% of those with long-standing flushing and atypical flushing (25%).[16]
signs of right heart failure
Includes peripheral edema and elevated jugular venous pressure.
Due to development of carcinoid heart disease.
cardiac murmurs
Thought to be due to serotonin and kinins leading to fibrosis of right-sided heart valves.
hepatomegaly
Suggests presence of liver metastases.
uncommon
wheeze
Occurs more commonly in patients with bronchial carcinoids than in midgut carcinoids. Incidence between 10% and 20%.[8]
pellagra
Uncommon, occurring in about 5% of cases.[8] Subclinical niacin deficiency is more common. Arises because tryptophan is required to produce niacin. Features of pellagra include diarrhea, dermatitis, and dementia.
abdominal masses
In midgut tumors, a desmoplastic reaction often develops in the surrounding tissue leading to the formation of a firm, palpable intra-abdominal mass.
Risk factors
weak
genetic multiple endocrine neoplasia type 1 (MEN-1) syndrome
Neuroendocrine tumors are associated with MEN-1 in about 10% of cases.[11] However, very few of these tumors cause carcinoid syndrome.
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