Emerging treatments
Surufatinib
Surufatinib, a tyrosine kinase inhibitor, was granted fast track designation by the Food and Drug Administration (FDA) for the treatment of advanced and progressive pancreatic and extrapancreatic neuroendocrine tumors (NET) based on two phase 3 trials carried out in China, and one phase 1 trial carried out in the US. In one China-based phase 3 trial of 198 adult patients with extrapancreatic NETs, progression-free survival was 9.2 months with surufatinib, compared with 3.8 months with placebo.[63] In 2022, the FDA rejected approval due to lack of multiregional clinical data (among other reasons). The manufacturer has since withdrawn its application for a marketing authorization in Europe. Surufatinib is approved in China.
Cabozanitib
Cabozantinib, a tyrosine kinase inhibitor, shows potential as a treatment option for patients with metastatic NETs, including VIPoma, although it has not been specifically tested in VIPoma. One phase 3 study in patients with both pancreatic and extra-pancreatic NETs reported that cabozantinib significantly delayed disease progression compared with placebo.[64] Clinical trials are recruiting or ongoing.
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