Treatment of Carcinoid of Small Intestine in Advanced Neuroendocrine Tumour Grade 3 with Ki-67 above 20%

This protocol covers the management of patients with advanced carcinoid of the small intestine classified as neuroendocrine tumour (NET) grade 3 — a presentation defined by a Ki-67 proliferative index above 20%. This is a rare and biologically high-grade scenario where treatment decisions require careful clinical judgement.

Clinical Scenario

First-line

Small intestine NETs (Si-NETs) are graded by proliferative activity. NET G3 is characterised by a Ki-67 index above 20% and a mitotic count above 20 per 2 mm². In the setting of advanced disease at this grade, the optimal treatment strategy is not firmly established, making a structured, evidence-informed approach essential.

Treatment Approach

For advanced Si-NET grade 3, systemic chemotherapy is the foundation of first-line treatment. Depending on patient-specific factors, additional targeted approaches may be considered in carefully selected cases. The complete protocol specifies the regimen options, patient selection criteria, and sequencing — details that go beyond this summary.

Instant Access to Structured Evidence-Based Regimens

References

DOI: 10.1111/jne.13423

NET G3 of small intestinal origin are rare and the best strategy in patients with advanced disease is unknown.
Si-NETs are graded as follows: NET G1 (Ki-67 <3%; <2 mitotic count in 2 mm²), NET G2 (Ki-67 3%–20%; mitotic count 2–20 in 2 mm²), NET G3 (Ki-67 >20%; mitotic count >20 in 2 mm²).
Despite having more evidence for pancreatic than for Si-NET, most centres use chemotherapy with temozolomide in combination with capecitabine or FOLFOX as a first-line therapy.
In carefully selected patients everolimus could be utilised.
In addition, in selected patients with homogeneous SST expression PRRT may also be an option.

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