Neuroendocrine Neoplasms clinical trials

Study of Mutation-Targeted Therapy With Sunitinib or Everolimus in People With Advanced Low- or Intermediate-Grade Neuroendocrine Tumors of the Gastrointestinal Tract and Pancreas With or Without Cytoreductive Surgery

Background: - Neuroendocrine tumors (NETs) come from cells of the hormonal and nervous systems. Some people have surgery to shrink the tumor. Sometimes the tumors come back. Researchers think that treatment with drugs based on knowing the defective gene might give better results. Objective: - To see if drugs selected based on the defective gene result in better tumor response. The drugs are Sunitinib and Everolimus. Eligibility: - People age 18 and older with an advanced low- or intermediate-grade gastrointestinal or pancreatic neuroendocrine tumor. Design: - Participants will be screened with: - Medical history - Physical exam - Scans - Blood, urine, and lab tests - The study team will see if participants should have surgery. - If yes, participants will: - Sign a separate consent - Have computed tomography (CT) scan before and after surgery - Have as much of the tumor removed as possible. A small piece will be tested for mutation type. - If no, participants will have a small piece of tumor removed for the testing. - If the surgery might cure them, the participant will leave the study. The other participants will be assigned to take either Sunitinib or Everolimus. - Participants will take their drug by mouth once a day. They will keep a medicine diary. Some will keep track of their blood pressure at least weekly. - Screening tests may be repeated at study visits. Participants also may have their heart evaluated. - About 30 days after the last day of their study drug, participants will have a follow-up visit that repeats the screening tests. - Participants will be contacted every 3 months after this visit.