Advanced small intestine neuroendocrine tumour (NET) with somatostatin receptor (SST) negative lesions represents a distinct clinical challenge. Because these tumours lack somatostatin receptor expression, standard receptor-targeted approaches are not appropriate, and other treatment strategies become necessary — particularly when locoregional therapy is also not an option.
Advanced small intestine neuroendocrine tumour with confirmed somatostatin receptor-negative tumour lesions. In this sub-population, SST-negative disease requires treatment pathways that differ from standard somatostatin analogue-based management.
DOI: 10.1111/jne.13423
In patients with advanced disease and SST negative lesions IFNα (RECOMMENDATION A-2b) or everolimus (RECOMMENDATION A-1b) can be used as first-line treatment if locoregional therapy is not an appropriate option.
Therefore, in patients with SST negative disease other treatment strategies are needed.
TKI may be considered based on accessibility and reimbursement after failure to approved therapies or in SST negative NET (RECOMMENDATION C-2b).
If accessible, a TKI may be considered (outside clinical trial setting) after failure of standard therapies (before chemotherapy) or in SST negative patients in case of intolerance to everolimus or IFN-α, or after failure of these therapies.
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