Pancreatic Endocrine Tumor - Market Insights, Epidemiology, and Market Forecast - 2034

Key Highlights:

• Pancreatic endocrine tumor or pancreatic neuroendocrine tumors (PNETs) are rare tumors, with an annual incidence of about 1 per 100,000 per year, and represent only 1-2% of pancreatic neoplasms.
• Non-functional pancreatic endocrine tumors are the most prevalent type of pancreatic endocrine tumors.
• Approximately 10% of all pancreatic neuroendocrine tumors arise in association with a hereditary syndrome, such as multiple endocrine neoplasia type 1 (MEN1), Von Hippel-Lindau (VHL) disease, neurofibromatosis type 1 (NF1), or tuberous sclerosis complex.
• Current standard treatment options include surgery (such as pancreatectomy or the Whipple procedure), peptide receptor radionuclide therapy (PRRT), chemotherapy, targeted therapies, somatostatin analogues, and radiofrequency ablation or transarterial chemoembolization.
• CABOMETYX (cabozantinib) (Exelixis), WELIREG (belzutifan) (Merck), and SUTENT (sunitinib) (Pfizer) are FDA-approved drugs for the treatment of patients with previously treated, unresectable, locally advanced or metastatic, well-differentiated pancreatic neuroendocrine tumors and extra-pancreatic neuroendocrine tumors (epNETs).
• The emerging pipeline for pancreatic endocrine tumor includes Zanzalintinib (Exelixis), RYZ101 (RayzeBio), Nab-sirolimus (Aadi Bioscience), and others.
• In June 2025, Exelixis announced that its partner Ipsen received approval from the European Commission (EC) for CABOMETYX for the treatment of adult patients with previously treated, unresectable or metastatic, well-differentiated pancreatic and extra-pancreatic neuroendocrine tumors. Further, the drug received approval from the US Food and Drug Administration in March 2025.

DelveInsight's "Pancreatic Endocrine Tumor- Market Insight, Epidemiology and Market Forecast - 2034" report delivers an in-depth analysis of pancreatic endocrine tumor epidemiology, market, and clinical development in pancreatic endocrine tumor. In addition to this, the report provides historical and forecasted epidemiology and market data as well as a detailed analysis of the pancreatic endocrine tumor market trends in the United States, EU4 (Germany, France, Italy, and Spain ), the United Kingdom, and Japan.

The Pancreatic Endocrine Tumor market report provides real-world prescription pattern analysis, emerging drugs assessment, market share, and uptake/adoption pattern of individual therapies, as well as historical and forecasted pancreatic endocrine tumor market size from 2020 to 2034 in 7MM. The report also covers current pancreatic endocrine tumor treatment practices/algorithms and unmet medical needs to curate the best opportunities and assess the market's underlying potential.

Geography Covered:

• The United States
• EU4 (Germany, France, Italy, and Spain) and the United Kingdom
• Japan

Pancreatic Endocrine Tumor Understanding and Treatment Algorithm

Pancreatic Endocrine Tumor Overview

Pancreatic Endocrine Tumor, also known as pancreatic neuroendocrine tumor (PNETs) or islet cell tumor is a rare type of cancer that develops from the hormone-producing cells (islet cells) in the pancreas. It can be either functional, secreting excess hormones that cause clinical syndromes such as insulinoma, gastrinoma, and glucagonoma, or non-functional, producing no active hormones and often presenting later due to non-specific symptoms. These tumors can be benign or malignant and may be associated with genetic syndromes like Multiple Endocrine Neoplasia type 1 (MEN1).

The symptoms of functional pancreatic endocrine tumor include diarrhea, malnutrition, fatigue, weakness, fluctuation in blood sugar level, and uncomfortable flushing of face and neck, wheezing and other carcinoid syndrome symptoms. However, the non-functional pancreatic endocrine tumor often does not cause any symptoms at early stages. Symptoms usually arise due to the tumor's size or spread (mass effect) and may include abdominal or belly pain, jaundice (yellowing of the skin and eyes), unexplained weight loss, nausea, presence of an abdominal lump, and loss of appetite.

Pancreatic Endocrine Tumor Diagnosis

The diagnosis of pancreatic endocrine tumor involves a multidisciplinary approach, including clinical evaluation, blood and urine tests, imaging tests, and tissue biopsy. The blood and urine tests to detect the level of hormones and genetic biomarkers, which help in characterizing functional tumors. Imaging tests such as abdominal CT scan, endoscopic ultrasound (EUS), MRI, and nuclear medicine scans like somatostatin receptor scintigraphy or PET scans localize and stage the tumor. EUS is particularly crucial for detecting small tumors and allows fine needle aspiration or biopsy for histopathological confirmation and grading. The tissue sampling via EUS-guided biopsy or surgery is essential to confirm the neuroendocrine nature of the tumor and to guide treatment planning. This integrated diagnostic evaluation ensures accurate tumor localization, assessment of hormonal activity, and staging for optimal management and prognosis.

Pancreatic Endocrine Tumor Treatment

The treatment of pancreatic endocrine tumor involves multiple approaches based on the tumor's type, location, stage, and the patient's overall health. The cornerstone treatment of pancreatic endocrine tumor is surgery for localized tumors, involving procedures such as pancreatectomy (distal or central) or the Whipple procedure based on tumor location. For advanced or metastatic disease, treatment includes peptide receptor radionuclide therapy (PRRT), targeted therapies such as sunitinib and everolimus, and somatostatin analogues like octreotide to control hormone-related symptoms and tumor growth. Further, treatment options for liver metastases include loco-regional treatments like radiofrequency ablation or transarterial chemoembolization. Chemotherapy is generally used for high-grade or progressive tumors but has limited efficacy. Overall, treatment is individualized based on tumor biology, extent, and patient condition to optimize outcomes and symptom control.

Pancreatic Endocrine Tumor Epidemiology

The pancreatic endocrine tumor epidemiology chapter in the report provides historical as well as forecasted epidemiology segmented as total incident cases of pancreatic endocrine tumor, gender-specific cases of pancreatic endocrine tumor, type-specific cases of pancreatic endocrine tumor, age-specific cases of pancreatic endocrine tumor, grade-specific cases of pancreatic endocrine tumor, function-specific cases of pancreatic endocrine tumor and total treated cases of pancreatic endocrine tumor in the 7MM covering the United States, EU4 (Germany, France, Italy, and Spain), and the United Kingdom, and Japan from 2020 to 2034.

• Pancreatic neuroendocrine tumors (pNETs) are rare. Less than 2% of all cancers found in the pancreas each year are pNETs.
• Most people with pNETs are older, with the average age at diagnosis being 60.
• In the United States, the incidence of localized pancreatic endocrine tumor is more prevalent than advanced or metastatic stage.
• Grade 1 PNETs represent the majority of cases, and PNETs occur more frequently in females.
• Approximately 70% of pancreatic endocrine tumor are non-functional, while the remaining 30% are functional and secrete hormones or peptides that cause clinical syndromes.

Pancreatic Endocrine Tumor Drug Chapters

The drug chapter segment of pancreatic endocrine tumor report encloses a detailed analysis of pancreatic endocrine tumor -marketed drugs and emerging pipeline drugs. It also deep dives into pancreatic endocrine tumor's pivotal clinical trial details, recent and expected market approvals, patent details, the latest news, and recent deals and collaborations.

Marketed Drugs

CABOMETYX (cabozantinib): Exelixis

CABOMETYX is a kinase inhibitor used for the treatment of patients aged 12 years and older with previously treated, unresectable, locally advanced or metastatic, well-differentiated pancreatic neuroendocrine tumors that are not surgically resectable. It works by inhibiting multiple tyrosine kinases involved in tumor growth and angiogenesis, helping to slow tumor progression.

In March 2025, the company announced that the US FDA had approved CABOMETYX for previously treated, unresectable, locally advanced or metastatic, well-differentiated pancreatic neuroendocrine tumors and extra-pancreatic neuroendocrine tumors (epNETs). The approval was based on positive results from the Phase III CABINET trial demonstrating significant improvement in progression-free survival for these patients.

WELIREG (belzutifan): Merck

WELIREG is an oral hypoxia-inducible factor inhibitor used for treatment of tumors associated with von Hippel-Lindau (VHL) disease, including pancreatic neuroendocrine tumors that do not require immediate surgery in adult patients. It works by inhibiting the hypoxia-inducible factor 2 alpha (HIF-2a), which plays a key role in tumor growth under low oxygen conditions, thereby contributing to tumor size reduction and slowing disease progression. The FDA approved belzutifan in August 2021 for VHL-associated pancreatic neuroendocrine tumors (pNETs) not requiring immediate surgery.

Zanzalintinib: Exelixis

Zanzalintinib is an investigational tyrosine kinase inhibitor for the treatment of Patients with locally advanced or metastatic neuroendocrine tumors, including pancreatic neuroendocrine tumor. Currently, the drug is being evaluated in Phase II/III clinical trial.

In June 2025, the company announced the initiation of STELLAR-311 Phase III pivotal trial in advanced pancreatic endocrine tumors. The primary endpoint of the trial is PFS per Response Evaluation Criteria in Solid Tumors (RECIST) 1.1 as assessed by Blinded Independent Central Review.

RYZ101 (225Ac-DOTATATE): RayzeBio/Bristol Myers Squibb

RYZ101 is a first-in-class, highly potent alpha-emitting radiopharmaceutical therapy being developed for treatment of patients with somatostatin receptor 2-expressing (SSTR2+) solid tumors, including pancreatic endocrine tumors (pNETs). It delivers targeted alpha radiation to tumors expressing SSTR2, leveraging the short path length and high energy of alpha particles to destroy tumor cells while sparing surrounding tissue. Currently, the drug is being evaluated in Phase III clinical trial.

In January 2025, RayzeBio presented the results from Phase Ib portion of the ACTION-1 Phase III trial of RYZ101 in SSTR2+ solid tumors, including pancreatic endocrine tumors (pNETs), and gastroenteropancreatic neuroendocrine tumors (GEP-NET) at the 2025 American Society of Clinical Oncology (ASCO) Gastrointestinal Cancers Symposium.

Drug Class Insights

Peptide receptor radionuclide therapy (PRRT)

Peptide Receptor Radionuclide Therapy (PRRT) is a targeted cancer therapy that utilizes a radioactive substance (radionuclide) attached to a peptide to deliver radiation directly to cancer cells. The radioactivity induces DNA damage, primarily by beta-particles, leading to tumor cell death while sparing most normal tissues. PRRT offers the advantage of targeted therapy, combining receptor-mediated delivery with molecular radiation, thereby improving treatment specificity and minimizing side effects. Further, this approach is particularly effective for treating advanced, unresectable, or metastatic neuroendocrine tumors that express specific peptide receptors, like somatostatin receptors.

Pancreatic Endocrine Tumor Market Outlook

The treatment of pancreatic endocrine tumors depends on tumor type, location, stage, and patient health. Surgery is the primary option for localized tumors, including pancreatectomy or the Whipple procedure. For advanced or metastatic cases, therapies include peptide receptor radionuclide therapy (PRRT) and somatostatin analogues such as octreotide to manage symptoms and tumor growth. Liver metastases may be treated with locoregional approaches like radiofrequency ablation or transarterial chemoembolization. The current FDA-approved therapies for the treatment of pancreatic endocrine tumors include CABOMETYX (cabozantinib) (Exelixis), WELIREG (belzutifan) (Merck), and SUTENT (sunitinib) (Pfizer).

The treatment landscape for pancreatic endocrine tumor is expected to advance significantly, with several innovative therapies currently in development, including Zanzalintinib (Exelixis), RYZ101 (RayzeBio), Nab-sirolimus (Aadi Bioscience), and others. These pipeline therapies have the potential to significantly transform the treatment landscape of pancreatic endocrine tumor and market dynamics in the coming years.

Pancreatic Endocrine Tumor Drug Uptake

This section focuses on the uptake rate of potential drugs expected to be launched in the market during 2025-2034. The landscape of pancreatic endocrine tumor treatment has experienced a profound transformation with the uptake of novel medicines. These innovative therapies are redefining standards of care.

Pancreatic Endocrine Tumor Pipeline Development Activities

The report provides insights into different therapeutic candidates in the marketed and emerging stages. It also analyses key players involved in developing targeted therapeutics.

Pipeline Development Activities

The report covers information on collaborations, acquisitions and mergers, licensing, and patent details for pancreatic endocrine tumor therapies.

KOL Views

To keep up with the real-world scenario in current and emerging market trends, we take opinions from Key Industry leaders working in the domain through primary research to fill the data gaps and validate our secondary research. Industry Experts were contacted for insights on the evolving treatment landscape, patient reliance on conventional therapies, patient therapy switching acceptability, and drug uptake, along with challenges related to accessibility, including Medical/scientific writers, Professors, and others.

DelveInsight's analysts connected with 20+ KOLs to gather insights; however, interviews were conducted with 15+ KOLs in the 7MM. Centers such as the University of Milan, the University of Texas Health Science Center at Houston, the European Institute of Oncology, etc., were contacted. Their opinion helps understand and validate current and emerging therapy treatment patterns or pancreatic endocrine tumor market trends.

Qualitative Analysis

We perform Qualitative and market Intelligence analysis using various approaches, such as SWOT analysis and Conjoint Analysis. In the SWOT analysis, strengths, weaknesses, opportunities, and threats in terms of gaps in disease diagnosis, patient awareness, physician acceptability, competitive landscape, cost-effectiveness, and geographical accessibility of therapies are provided.

Conjoint Analysis analyzes multiple approved and emerging therapies based on relevant attributes such as safety, efficacy, frequency of administration, route of administration, and order of entry. Scoring is given based on these parameters to analyze the effectiveness of therapy.

In efficacy, the trial's primary and secondary outcome measures are evaluated; for instance, in event-free survival, one of the most important primary outcome measures is event-free survival and overall survival.

Further, the therapies' safety is evaluated wherein the acceptability, tolerability, and adverse events are majorly observed, and it sets a clear understanding of the side effects posed by the drug in the trials. In addition, the scoring is also based on the probability of success, and the addressable patient pool for each therapy. According to these parameters, the final weightage score and the ranking of the emerging therapies are decided.

Market Access and Reimbursement

Reimbursement may be referred to as the negotiation of a price between a manufacturer and a payer that allows the manufacturer access to the market. It is provided to reduce the high costs and make the essential drugs affordable. Health technology assessment (HTA) plays an important role in reimbursement decision-making and recommending the use of a drug. These recommendations vary widely throughout the seven major markets, even for the same drug. In the US healthcare system, both Public and Private health insurance coverage are included. Also, Medicare and Medicaid are the largest government-funded programs in the US. The major healthcare programs, including Medicare, Medicaid, Health Insurance Program (CHIP), and the state and federal health insurance marketplaces, are overseen by the Centers for Medicare & Medicaid Services (CMS). Other than these, Pharmacy Benefit Managers (PBMs) and third-party organizations that provide services and educational programs to aid patients are also present.

The report further provides detailed insights on the country-wise accessibility and reimbursement scenarios, cost-effectiveness scenario of currently used therapies, programs making accessibility easier and out-of-pocket costs more affordable, insights on patients insured under federal or state government prescription drug programs, etc.

Scope of the Report:

• The report covers a segment of key events, an executive summary, a descriptive overview of pancreatic endocrine tumor, explaining their causes, signs and symptoms, pathogenesis, and currently available therapies.
• Comprehensive insight has been provided into the epidemiology segments and forecasts, the future growth potential of the diagnosis rate, and treatment guidelines.
• Additionally, an all-inclusive account of both the current and emerging therapies, along with the elaborative profiles of late-stage and prominent therapies, will have an impact on the current treatment landscape.
• A detailed review of the pancreatic endocrine tumor market, historical and forecasted market size, market share by therapies, detailed assumptions, and rationale behind our approach is included in the report, covering the 7MM drug outreach.
• The report provides an edge while developing business strategies, by understanding trends, through SWOT and conjoint analysis and expert insights/KOL views, patient journey, and treatment preferences that help in shaping and driving the 7MM pancreatic endocrine tumor market.

Pancreatic Endocrine Tumor Report Insights

• Patient Population
• Therapeutic Approaches
• Pancreatic Endocrine Tumor Pipeline Analysis
• Pancreatic Endocrine Tumor Market Size and Trends
• Existing and Future Market Opportunity

Pancreatic Endocrine Tumor Report Key Strengths

• Ten-Year Forecast
• 7MM Coverage
• Pancreatic Endocrine Tumor Epidemiology Segmentation
• Key Cross Competition
• Conjoint analysis
• Drugs Uptake and Key Market Forecast Assumptions

Pancreatic Endocrine Tumor Report Assessment

• Current Treatment Practices
• Unmet Needs
• Pipeline Product Profiles
• Market Attractiveness
• Qualitative Analysis (SWOT and Conjoint)

FAQs:

• What was the pancreatic endocrine tumor total market size, the market size by therapies, market share (%) distribution in 2020, and what would it look like in 2034? What are the contributing factors for this growth?
• At what CAGR is, pancreatic endocrine tumor market is expected to grow at the 7MM level during the study period (2020-2034)?
• How is Japan's pancreatic endocrine tumor competitive landscape evolving?
• How will upcoming emerging therapies are going to impact CABOMETYX's market share?
• What are the disease risks, burdens, and unmet needs of pancreatic endocrine tumor?
• What will be the growth opportunities across the 7MM with respect to the patient population pertaining to pancreatic endocrine tumor?
• What is the historical and forecasted pancreatic endocrine tumor patient pool in the United States, EU4 (Germany, France, Italy, and Spain), the United Kingdom, and Japan?
• What factors are affecting the increase in the diagnosis of symptomatic cases?
• What are the current options for the treatment of pancreatic endocrine tumor? What are the current treatment guidelines for the treatment of pancreatic endocrine tumor in the US and Europe?
• How many companies are developing therapies for the treatment of pancreatic endocrine tumor?
• Which key designations have been granted for the emerging therapies for pancreatic endocrine tumor?
• What is the cost burden of approved therapies on the patient?
• Patient acceptability in terms of preferred treatment options as per real-world scenarios?
• What are the country-specific accessibility issues of expensive, recently approved therapies? Focus on reimbursement policies.

Reasons to Buy:

• The report will help in developing business strategies by understanding the latest trends and changing treatment dynamics and driving factor for pancreatic endocrine tumor market.
• Insights on patient share/disease burden, evolution in diagnosis, and factors contributing to the change in the epidemiology of the disease during the forecast years.
• Understand the existing market opportunities in varying geographies and the growth potential over the coming years.
• Distribution of historical and current patient share based on real-world prescription data along with reported sales of approved products in the US, EU4 (Germany, France, Italy, and Spain), the United Kingdom, and Japan.
• Identifying upcoming players in the market will help devise strategies to help get ahead of competitors.
• Highlights of access and reimbursement policies of approved therapies, barriers to accessibility of expensive off-label therapies, and patient assistance programs.
• To understand KOLs' perspectives around the accessibility, acceptability, and compliance-related challenges of existing treatment to overcome barriers in the future.
• Detailed insights on the unmet needs of the existing market so that the upcoming players can strengthen their development and launch strategy.