Systemic Therapy for Advanced and Metastatic Disease

Somatostatin analogues may be effective in reducing the symptoms of functional tumors.[14]

Chemotherapy using drugs such as the following, either alone or in combination, has been shown to have antitumor effects, but evidence is weak or conflicting regarding the impact of chemotherapy on overall survival:[15-17]

A variety of systemic agents have shown biological or palliative activity, including:[5,18]

Nearly all of the evidence of activity is derived from case series.[Level of evidence C3] However, there are ongoing placebo-controlled randomized trials of everolimus [19] and sunitinib [20] that have been reported in abstract form showing an increase in progression-free survival in each case.[Level of evidence B1]

Favorable responses have been reported in patients with advanced progressive pancreatic NETs after treatment with several radiolabeled somatostatin analogues in which the analogues octreotide, octreotate, lanreotide, or edotreotide are stably attached to the radionuclides indium In 111, yttrium Y 90, or lutetium Lu 177.[21-23] The relative efficacy of these various compounds is unknown. Study designs have been limited to case series with tumor response, biochemical response, or symptom control as the measure of efficacy.[Level of evidence C3]

As noted in each of the clinical situations, there is a paucity or absence of high-level evidence and a need for randomized controlled trials.[5]