This protocol applies to patients with carcinoid tumor of thymus presenting with carcinoid syndrome in whom first-line long-acting somatostatin analogue (SSA) therapy has not produced significant improvement — defining the threshold for escalation to the next treatment step.
Carcinoid tumor of thymus with concurrent carcinoid syndrome. First-line symptomatic management was initiated with long-acting SSAs, with short-acting SSAs given intravenously in the perioperative setting. Despite this, the primary goal — significant improvement of carcinoid syndrome — was not achieved.
Previous treatment: Long-acting SSAs as first-line symptomatic therapy. Escalation to this protocol is triggered by failure to achieve significant improvement of carcinoid syndrome under that regimen.
In refractory carcinoid syndrome, the protocol distinguishes between different disease trajectories and applies a structured set of options accordingly. The approach involves both disease-directed and symptom-directed interventions, selected on the basis of how the disease is progressing. The full decision framework and the specific options within each pathway are available via the link below.
DOI: 10.1016/j.annonc.2021.01.003
We recommend long-acting SSAs as first-line symptomatic treatment of CS [V, B].
In patients with refractory CS, a variety of options exist but there is no consensus on the best strategy, due to the lack of specific LC studies.
Only telotristat ethyl has been approved by the European Medicines Agency (EMA) and the United States Food and Drug Administration (FDA) for the treatment of the diarrhoea of refractory CS.
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