Carcinoid syndrome
ICD-10 E34.0 · ICD-11 5B10

Carcinoid Syndrome When SSA Dose-Escalation and Telotristat Ethyl Have Not Controlled Refractory Diarrhoea

Clinical Scenario

This protocol addresses patients with carcinoid syndrome who have progressed through an intensified first-line medical regimen and continue to have refractory symptoms — particularly those with liver-predominant metastatic disease driving persistent hormonal secretion.

Previous Treatment Line — Goals Not Achieved

The prior regimen — dose-escalation of SSA (octreotide LAR or lanreotide Autogel) with or without the addition of telotristat ethyl — aimed to achieve:

Improvement of refractory CS-associated diarrhoea and reduction in urinary 5-HIAA levels.

Failure to reach these targets is the trigger for escalation to the approach described in this protocol.

Next-Step Approach (Partial Overview)

Management shifts towards liver-directed loco-regional intervention, targeting the hepatic metastatic burden responsible for continued CS symptoms. Surgical evaluation is incorporated into the decision pathway. SSA is maintained throughout.

The complete protocol — covering specific modality selection, sequencing, and eligibility criteria — is available via the link below.

Clinical goal: Control of carcinoid syndrome symptoms.

Instant Access to Structured Evidence-Based Regimens

References

DOI: 10.1111/jne.13146

Loco-regional therapies may be considered for patients with predominant liver inoperable metastases, requiring CS control.

Hepatic trans-arterial embolisation (TAE), trans-arterial chemoembolisation (TACE) or trans-arterial radioembolisation/selective internal radiotherapy (TARE/SIRT) controls CS symptoms in up to 75% cases and can be repeated if there is preserved liver function.

Hepatic resection should be applied with curative intent (R0 resection of metastatic lesions) or considered for symptom relief as cytoreductive (debulking) surgery, based on tumour operability/metastatic type.

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