This page covers the first-line management approach for small intestine neuroendocrine tumour (Si-NET) presenting with carcinoid syndrome — the distinct clinical phenotype characterised by secretory diarrhoea and cutaneous flushing.
Patients with Si-NET who develop carcinoid syndrome (CS) — manifesting as diarrhoea and flushing — require a treatment strategy that differs from that used in non-functioning Si-NET. The syndrome arises from secretion of biologically active compounds and may also present acutely during surgery for intestinal obstruction or bleeding.
The cornerstone of first-line management in this setting is a specific class of hormonal therapy directed at suppressing the secretory symptoms of carcinoid syndrome. Rescue therapy for breakthrough symptomatic episodes is also part of the strategy.
The primary clinical objectives are symptomatic improvement of diarrhoea and flushing, along with reduction of 5-HIAA levels — a biochemical marker used to monitor treatment response in patients with CS.
It is important to note that patients suffering from CS are treated differently from patients with non-functioning Si-NET.
Some patients present with specific symptoms of the carcinoid syndrome (CS) due to secretion of biologically active compounds such as serotonin and tachykinins or are discovered during surgery for intestinal obstruction or bleeding.
Addition of short acting octreotide s.c. to depot preparations of SSA as rescue therapy for breakthrough symptoms may also be an option.
5-HIAA, either measured in blood or 24 h urine, should be monitored in Si-NET patients with suspected CS and to assess treatment response in patients with CS (RECOMMENDATION A-2a).
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