The full text of
this review is available only by subscription, but the audio
summary is open access.
Here are a few key
points:
Carcinoid tumors
(CT) tend to grow slowly and remain asymptomatic for long periods.
Carcinoid syndrome (CS), the clinical state resulting from release of
mediators by the tumor, generally does not occur until it
metastasizes to the liver, though there are exceptions to this rule.
CS consists of vasomotor disturbances, flushing, bronchospasm and
diarrhea and is primarily mediated by serotonin although other
mediators including kinins, histamine and prostaglandins are
involved.
Carcinoid heart
disease (CHD) is a valvulopathy (most frequently the tricuspid,
followed in order of frequency by the pulmonic and then the left
sided valves) caused by an inflammatory and fibroproliferative
response of valvular endocardium to the circulating mediators, most
notably serotonin. Regurgitation is the primary lesion affecting the
valves although stenosis can occur to a lesser degree.
It is estimated that
50% of patients with CS go on to develop CHD though that number may
be decreasing with improvements in recognition and treatment of CS.
Once CHD develops it tends to progress rapidly and worsens the
prognosis of patients with CS.
Transcatheter
embolization and surgical debulking of liver metastases are indicated
in some patients but become less viable options if hepatic congestion
has developed due to the risk of acute liver failure and bleeding.
Valve replacement
may improve the outlook for selected patients.
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