This protocol covers the management of pulmonary neuroendocrine tumors in patients presenting with carcinoid syndrome — a clinical scenario defined by hormonal hypersecretion of serotonin and other compounds from the tumor.
A subset of pulmonary neuroendocrine tumor cases manifest with carcinoid syndrome driven by serotonin and other compound secretions. This pattern of hormonal activity is a defining feature of this patient subgroup and directly informs treatment selection.
In refractory carcinoid syndrome, treatment strategy is shaped by the pace of disease progression. For slowly progressive presentations, pharmacological options include agents specifically targeting serotonin-mediated symptoms — among them a therapy with regulatory approval from both the EMA and FDA for this indication — along with somatostatin-based approaches. More significantly progressive disease is addressed by a distinct set of systemic interventions. The full protocol details the complete range of options and how disease tempo guides selection between them.
DOI: 10.1016/j.annonc.2021.01.003
A minority of cases present with symptoms related to hormonal hypersecretion, including carcinoid syndrome (CS), due to serotonin and other compound secretions, Cushing's syndrome (CuS), due to adrenocorticotropic hormone (ACTH) secretion and acromegaly, due to growth hormone-releasing hormone (GHRH) secretion.
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.
Among second-line options described in Figure 3 and Supplementary Material, available at https://doi.org/10.1016/j.annonc.2021.01.003, 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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