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Pancreatic Neuroendocrine Tumor (NET)
Targeted drugs work differently from standard chemotherapy (chemo) drugs. These drugs target specific parts of cancer cells.
The targeted drugs used to treat pancreatic neuroendocrine tumors (NETs) by blocking angiogenesis (the growth of new blood vessels that nourish cancers) or other important proteins in cancer cells that help them grow.
Sunitinib blocks several tyrosine kinases and attacks new blood vessel growth. It has been shown to help slow tumor growth. This drug is a pill taken once a day.
The most common side effects are tiredness (fatigue), nausea, vomiting, diarrhea, constipation, abdominal pain, mouth sores, problems breathing, cough, and changes in skin or hair color. Other possible effects include high blood pressure, heart problems, bleeding, hand-foot syndrome (redness, pain, and skin peeling of the palms of the hands and the soles of the feet), and low thyroid hormone levels.
Everolimus blocks a protein known as mTOR, which normally helps cells grow and divide. Everolimus has been shown to help treat advanced pancreatic NETs. Everolimus is a pill taken once a day.
Common side effects of this drug include mouth sores, infections, loss of appetite, diarrhea, tiredness, cough, and increases in blood sugar and cholesterol levels. A less common but serious side effect is damage to the lungs, which can cause shortness of breath (dyspnea) or other problems.
Belzutifan is a type of drug known as a HIF inhibitor. It blocks a protein called hypoxia-inducible factor 2 alpha (HIF-2a), which is involved in how a cell responds to low oxygen levels (hypoxia) and the formation of new blood vessels in tumors. This drug is taken as pills, typically once a day.
Belzutifan can be used in people with von Hippel-Lindau (VHL) disease who have an advanced pancreatic NET.
Common side effects of this drug include low red blood cell counts (anemia), feeling tired and/or dizzy, nausea, headache, increased blood sugar levels, and changes in lab tests showing the drug might be affecting the kidneys. Less common but more serious side effects can include very low red blood cell counts (severe anemia), which might require blood transfusions, and low oxygen levels in the body, for which you might need oxygen therapy or even be admitted to the hospital.
To learn more about how targeted drugs are used to treat cancer, see Targeted Cancer Therapy.
To learn about some of the side effects listed here and how to manage them, see Managing Cancer-related Side Effects.
The American Cancer Society medical and editorial content team
Our team is made up of doctors and oncology certified nurses with deep knowledge of cancer care as well as editors and translators with extensive experience in medical writing.
Jonasch E, Donskov F, Iliopoulos O, et al. Belzutifan for renal cell carcinoma in von Hippel-Lindau disease. NEJM 2021;385:2036-2046.
Ma ZY, Gong YF, Zhuang HK, Zhou ZX, Huang SZ, Zou YP, Huang BW, Sun ZH, Zhang CZ, Tang YQ, Hou BH. Pancreatic neuroendocrine tumors: A review of serum biomarkers, staging, and management. World J Gastroenterol. 2020 May 21;26(19):2305-2322. doi: 10.3748/wjg.v26.i19.2305. PMID: 32476795; PMCID: PMC7243647.
Mpilla GB, Philip PA, El-Rayes B, Azmi AS. Pancreatic neuroendocrine tumors: Therapeutic challenges and research limitations. World J Gastroenterol. 2020 Jul 28;26(28):4036-4054. doi: 10.3748/wjg.v26.i28.4036. PMID: 32821069; PMCID: PMC7403797.
National Cancer Institute. Physician Data Query (PDQ). Pancreatic Neuroendocrine Tumors (Islet Cell Tumors) Treatment – Patient Version. 10/7/22. Accessed at https://www.cancer.gov/types/pancreatic/patient/pnet-treatment-pdq on August 4, 2024.
National Comprehensive Cancer Network. NCCN Clinical Practice Guidelines in Oncology: Neuroendocrine and Adrenal Tumors. V.2.2024. Accessed at https://www.nccn.org/professionals/physician_gls/pdf/neuroendocrine.pdf on August 4, 2024.
Pelle E, Al-Toubah T, Morse B, Strosberg J. Belzutifan in a Patient With VHL-Associated Metastatic Pancreatic Neuroendocrine Tumor. J Natl Compr Canc Netw. 2022 Dec;20(12):1285-1287. doi: 10.6004/jnccn.2022.7047. PMID: 36509068.
Raymond E, Dahan L, Raoul JL, et al. Sunitinib malate for the treatment of pancreatic neuroendocrine tumors. NEJM. 2011;364:501−513.
Schneider DF, Mazeh H, Lubner SJ, Jaume JC, Chen H. Chapter 71: Cancer of the endocrine system. In: Niederhuber JE, Armitage JO, Dorshow JH, Kastan MB, Tepper JE, eds. Abeloff’s Clinical Oncology. 5th ed. Philadelphia, Pa. Elsevier: 2014.
Yao JC, Evans DB. Chapter 85: Pancreatic neuroendocrine tumors. In: DeVita VT, Lawrence TS, Rosenberg SA, eds. DeVita, Hellman, and Rosenberg’s Cancer: Principles and Practice of Oncology. 10th ed. Philadelphia, Pa: Lippincott Williams & Wilkins; 2015.
Yao JC, Shah MH, Ito T, et al. Everolimus for advanced pancreatic neuroendocrine tumors. NEJ M. 2011;364:514−523.
Last Revised: August 22, 2024
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