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New and Emerging Treatment Options for Gastroenteropancreatic Neuroendocrine Tumors
Alexandria T Phan, Pamela L Kunz1, Diane L Reidy-Lagunes
1Stanford University School of Medicine, Stanford, California.
Abstract:
Gastroenteropancreatic neuroendocrine tumors (GEP-NETs) are rare, generally indolent neoplasms that can arise throughout the gastrointestinal system. Some GEP-NETs, known as functional, secrete hormones that can lead to a complex of symptoms. Classical carcinoid syndrome is associated with flushing, diarrhea, bronchospasm, and symptoms of valvular heart disease. GEP-NETs are classified according to the primary tumor site, functionality of the disease, and histology. Treatment is guided by the resectability of the tumor, the location and extent of metastases, and the presence of clinical symptoms. Typically, first-line treatment of patients with unresectable disease includes the use of somatostatin analogs, such as octreotide LAR depot or lanreotide depot/autogel, which was recently approved by the US Food and Drug Administration for treatment of GEP-NETs. Somatostatin analogs can improve the severe diarrhea/flushing episodes that may be associated with metastatic carcinoid tumors. For patients with pancreatic NETs, additional approved treatment options include the targeted agents everolimus and sunitinib, which have demonstrated antitumor activity. Chemotherapy may also have a selective role, particularly in pancreatic NETs. Localized approaches, including cytoreductive surgery, hepatic arterial embolization, and ablative therapies, may be used for palliative treatment in patients with liver metastases.
Insights
Gastroenteropancreatic neuroendocrine tumors (GEP-NETs) are rare. Treatment for unresectable GEP-NETs includes somatostatin analogs, targeted agents like everolimus and sunitinib for pancreatic NETs, and localized therapies.
Area of Science:
- Oncology
- Gastroenterology
- Endocrinology
Background:
- Gastroenteropancreatic neuroendocrine tumors (GEP-NETs) are rare neoplasms with varying clinical presentations.
- Functional GEP-NETs can secrete hormones, leading to syndromes like carcinoid syndrome (flushing, diarrhea, bronchospasm, cardiac issues).
- Classification relies on primary site, functionality, and histology, guiding treatment decisions.
Purpose of the Study:
- To outline the classification and current treatment strategies for GEP-NETs.
- To highlight therapeutic options for unresectable GEP-NETs, including medical and localized approaches.
- To discuss specific treatments for pancreatic neuroendocrine tumors (PNETs).
Main Methods:
- Review of current treatment guidelines and therapeutic options for GEP-NETs.
- Discussion of first-line treatments such as somatostatin analogs (octreotide LAR, lanreotide).
- Exploration of additional therapies for PNETs (everolimus, sunitinib, chemotherapy) and palliative local treatments.
Main Results:
- Somatostatin analogs are a standard first-line treatment for unresectable GEP-NETs, managing symptoms like diarrhea and flushing.
- Everolimus and sunitinib show antitumor activity in pancreatic NETs.
- Localized therapies (surgery, embolization, ablation) offer palliative care for liver metastases.
Conclusions:
- Treatment for GEP-NETs is individualized based on resectability, metastatic extent, and symptoms.
- Somatostatin analogs, targeted agents, chemotherapy, and local therapies form a multi-modal approach to GEP-NET management.
- Ongoing research and drug approvals continue to expand treatment options for these rare tumors.
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