Inclusion Criteria:

- Histopathologically-confirmed, well-differentiated metastatic neuroendocrine tumor

- Documented history of carcinoid syndrome and currently experiencing ≥4 bowel
movements per day during the Run-in period

- Currently receiving stable-dose somatostatin analog (SSA) therapy

- Minimum dose of LAR or depot SSA therapy

- Octreotide LAR at 30 mg every 4 weeks

- Lanreotide Depot at 120 mg every 4 weeks

- Patients who cannot tolerate SSA therapy at a level indicated above will be
allowed to enter at their highest tolerated dose

- Ability and willingness to provide written informed consent

Exclusion Criteria:

- Presence of diarrhea attributed to any condition(s) other than carcinoid syndrome

- Karnofsky Performance status ≤60%

- Treatment with any tumor directed therapy, including interferon, chemotherapy, mTOR
inhibitors ≤4 weeks prior to Screening, or hepatic embolization, radiotherapy,
radiolabelled SSA, and/or tumor debulking ≤12 weeks prior to Screening

- History of short bowel syndrome (SBS)

- Clinically significant cardiac arrhythmia, bradycardia, tachycardia that would
compromise patient safety or the outcome of the study, or QTcF >450 ms

- Previous exposure to telotristat etiprate