Carcinoid of small intestine
ICD-10 D37.2 · ICD-11 2B80.21

Treatment of Advanced Unresectable Small Intestine Neuroendocrine Tumour (Somatostatin Receptor Positive, Grade 1 or Grade 2, Without Carcinoid Syndrome)

This protocol addresses the management of advanced, unresectable small intestine neuroendocrine tumour (Si-NET) that is grade 1 or grade 2, confirmed somatostatin receptor positive, and presents without carcinoid syndrome.

Clinical scenario: Advanced unresectable Si-NET, grade 1 or grade 2, somatostatin receptor positive, without carcinoid syndrome. Receptor status is a central determinant of therapy selection and sequencing throughout the treatment course.

Treatment approach

In the setting of somatostatin receptor positive Si-NET, long-acting somatostatin analogues form the preferred initial approach. When standard lines have been exhausted, selected later-line options exist — including rechallenge with a peptide receptor radionuclide therapy approach, escalated somatostatin analogue dosing, or alternative systemic agents — though the appropriate choice depends on prior treatment history and tolerability. Full sequencing and criteria are in the structured protocol.

Instant Access to Structured Evidence-Based Regimens

References

DOI: 10.1111/jne.13423

In the presence of advanced unresectable Si-NET G1 or G2 long-acting SSA (either lanreotide or octreotide) is the preferred first-line treatment (RECOMMENDATION A-1b).

The recommended second-line treatment in the presence of SST-positive Si-NET is PRRT (RECOMMENDATION A-1b), followed by everolimus (RECOMMENDATION A-1b).

Rechallenge PPRT (RECOMMENDATION C-4) and high dose SSA (RECOMMENDATION B-2) may be considered in selected scenarios.

TKI may be considered based on accessibility and reimbursement after failure to approved therapies or in SST negative NET (RECOMMENDATION C-2b).

If utilised, rechallenge with PRRT would be delivering two more cycles of PRRT, and this could be repeated again in the case of progression after another >12 months and if the treatment was well tolerated.

When doing so, temozolomide and capecitabine or FOLFOX are the recommended schedules to use.

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