Related Experiment Video
Updated: Jul 16, 2026

Establishment and Characterization of Small Bowel Neuroendocrine Tumor Spheroids
Published on: October 14, 2019
Neuroendocrine tumors. Molecular targeted therapy for carcinoid and islet-cell carcinoma
1Department of Gastrointestinal Medical Oncology, The University of Texas M. D. Anderson Cancer Center, Houston, TX 77030, USA. jyao@mail.mdanderson.org
Abstract:
Carcinoid and islet-cell carcinoma are often also known as low-grade neuroendocrine carcinomas. They are often slow-growing but can be resistant to standard therapy. While somatostatin analogues are often used to control hormonal syndromes, there is currently no therapy approved in the US for control of carcinoid tumor growth. For islet-cell carcinoma, streptozocin-based chemotherapy may induce tumor shrinkage, but second-line option are limited. This chapter reviews the molecular biology of neuroendocrine tumors, including the roles of MENIN, TSC2, NF-1, vHL, p53, bcl-2, bax, VEGF, IGF, PDGF, EGFR, and mTOR. Recently, there has been interest in developing molecularly targeted therapy for this group of diseases. Phase-II studies with imatinib, bevacizumab, sunitinib, gefitnib, temsirolimus, and everolimus (RAD001) have completed accrual. Encouraging results have been observed in studies with VEGF and mTOR inhibitors. Phase-III study of bevacizumab is planned in the US. Large-scale multinational phase-II and -III studies of everolimus are under way.
Insights
Low-grade neuroendocrine tumors, including carcinoid and islet-cell carcinomas, show promise with molecularly targeted therapies. VEGF and mTOR inhibitors demonstrate encouraging results, with ongoing clinical trials for advanced treatments.
Area of Science:
- Oncology
- Molecular Biology
- Endocrinology
Background:
- Carcinoid and islet-cell carcinomas are low-grade neuroendocrine tumors.
- These tumors are often slow-growing and can be resistant to conventional therapies.
- Current treatments for carcinoid tumor growth are limited, and second-line options for islet-cell carcinoma are scarce.
Purpose of the Study:
- To review the molecular biology of neuroendocrine tumors.
- To discuss the potential of molecularly targeted therapies for these diseases.
Main Methods:
- Review of molecular biology, including genes like MENIN, TSC2, NF-1, vHL, p53, bcl-2, bax, VEGF, IGF, PDGF, EGFR, and mTOR.
- Analysis of Phase-II study data for targeted agents: imatinib, bevacizumab, sunitinib, gefitinib, temsirolimus, and everolimus (RAD001).
Main Results:
- Encouraging results observed with vascular endothelial growth factor (VEGF) and mammalian target of rapamycin (mTOR) inhibitors.
- Phase-II studies with targeted agents have completed accrual, providing preliminary efficacy data.
Conclusions:
- Molecularly targeted therapies, particularly VEGF and mTOR inhibitors, represent a promising avenue for treating neuroendocrine tumors.
- Ongoing Phase-II and -III clinical trials, including those for bevacizumab and everolimus, are crucial for establishing new treatment standards.
Related Concept Videos
Targeted Cancer Therapies
There are several types of targeted therapies against specific...
Targeted Cancer Therapies
There are several types of targeted therapies against specific...
Tumor Immunotherapy
Chemotherapy-Induced Nausea and Vomiting: Neurokinin-1 Receptor Antagonists
Inhibition of Cdk Activity
Combination Therapies and Personalized Medicine
The combination of the drug acetazolamide and sulforaphane is a good example of combination therapy to treat cancer. The cells in the interior of a large tumor often die due to the hypoxic and...