Background: Neuroendocrine tumors (NETs) are highly vascular neoplasms overexpressing VEGF as well as VEGFR. Axitinib is an inhibitor of receptor tyrosine kinases with picomolar potency against VEGFR-1, -2 and -3 and nanomolar potency against PDGFR-β. Methods: We performed a phase II trial of axitinib 5 mg twice daily in patients with unresectable or metastatic low to intermediate grade carcinoid tumors. Prior antiangiogenic therapy with a dedicated VEGF pathway inhibitor was not permitted. The primary endpoints were PFS and 12-month PFS rate. H₀= 12 mo PFS rate of 36% (corresponding to median PFS of 8.1 months); H_a= 12 mo PFS rate of 56.5% (corresponding to median PFS of 14.6 months). Preliminary findings are reported. Results: 30 patients were enrolled and assessable for toxicity; 22 were assessable for response. Primary sites included small intestine (19 patients), lung (3), unknown (3), colon (2), rectum (2), and thymus (1). 21 patients had low-grade and 9 patients had intermediate-grade tumors. Carcinoid syndrome was diagnosed in 16/30 patients. Median TTF was 8.99 months (SD ±7.18) and the 12 mo PFS rate was 65% (SD ±13%). Median PFS not yet determined due to small number of events. The 1-year OS rate was 93% (SD±4.9%). Best radiographic response was PR in 1/30 (3.3%) and stable disease in 21/30 (70%); 8/30 patients (27%) unevaluable due to toxicity (4/30) and consent withdrawn (4/30). Among 25 patients with baseline elevated chromogranin A levels, 4 (16%) experienced major reductions ( > 50%) of the tumor marker. 90% of patients experienced hypertension (any grade). Grade 3 and 4 hypertension were seen in 18 (60%) and 2 (7%) patients respectively. Hypertension led to treatment discontinuation in two cases, however, axitinib interruption prompted a fast recovery without sequelae. Conclusions: The 12 mo PFS rate associated with axitinib in advanced carcinoid tumors appears promising when compared to results observed in phase II studies of other antiangiogenic TKIs such as sunitinib or pazopanib. Although associated hypertension is common, axitinib treatment was overall well tolerated. Clinical trial information: NCT01435122