Neuroendocrine carcinoma of pancreas

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1Active trials29Specialists8Treatment centers

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UniteRare data is sourced from FDA.gov, ClinicalTrials.gov, Orphanet, OMIM, and NORD.
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Overview

Neuroendocrine carcinoma of the pancreas (also called pancreatic neuroendocrine carcinoma, or NEC of the pancreas) is a rare and aggressive type of cancer that starts in the hormone-producing cells of the pancreas. The pancreas has two main jobs: helping with digestion and making hormones like insulin that control blood sugar. Neuroendocrine carcinomas arise from the cells that make these hormones, but unlike the more common and slower-growing pancreatic neuroendocrine tumors (PNETs), neuroendocrine carcinomas grow and spread quickly. They are considered high-grade, meaning the cancer cells look very abnormal and divide rapidly. This cancer can cause a wide range of symptoms depending on whether it releases hormones into the bloodstream. Some tumors are 'functional' and produce hormones that cause noticeable symptoms like low blood sugar or diarrhea. Others are 'non-functional' and may not cause symptoms until the tumor grows large or spreads. Common symptoms include abdominal pain, unexplained weight loss, nausea, and fatigue. Treatment usually involves a combination of surgery (when possible), chemotherapy, and sometimes targeted therapies. Because this cancer is aggressive and often diagnosed at a late stage, treatment focuses on controlling the disease and improving quality of life. A team of specialists works together to create a personalized plan for each patient.

Also known as:

Key symptoms:

Abdominal or upper belly painUnexplained weight lossNausea and vomitingLoss of appetiteFatigue and weaknessJaundice (yellowing of the skin or eyes)Episodes of low blood sugar (shakiness, sweating, confusion)Diarrhea or changes in bowel habitsNew or worsening diabetesA lump or mass felt in the abdomenBack painFlushing or redness of the face and neck

Inheritance

Sporadic

Usually appears on its own, not inherited from a parent

Age of Onset

Adult

Begins in adulthood (age 18 or older)

Orphanet ↗NORD ↗

FDA & Trial Timeline

10 events
Oct 2025FTT PET/CT in Pancreatic Neuroendocrine Tumors

Abramson Cancer Center at Penn Medicine — PHASE1

TrialRECRUITING
Sep 2025Efficacy and Safety Evaluation of Tarlatamab in Advanced Extrapulmonary Neuroendocrine Carcinoma Patients

Inkeun Park — PHASE2

TrialRECRUITING
Jul 2024A Trial to Assess Efficacy, Safety, Pharmacokinetics of Octreotide Subcutaneous Injection in Patients With Gastroentero-pancreatic Neuroendocrine Tumor (GEP-NET)

CSPC ZhongQi Pharmaceutical Technology Co., Ltd. — PHASE2

TrialRECRUITING
Jun 2024Using an End-of-life Conversation Game to Engage Patients With Cancer in Advance Care Planning: Phase 2

Milton S. Hershey Medical Center — NA

TrialACTIVE NOT RECRUITING
May 2024Safety and Efficacy of KLS-1 Monotherapy in Malignant Neoplasms

Vector Vitale LLC — PHASE1, PHASE2

TrialRECRUITING
Oct 2023Leflunomide or Combination of MEK Inhibitor and Hydroxychloroquine for Refractory Patients With RAS Mutations

N.N. Petrov National Medical Research Center of Oncology — PHASE2

TrialRECRUITING
Mar 2023Generation of Organoids of Neuroendocrine Neoplasms of the Gastro-Entero-Pancreatic Tract Obtained From Patients Undergoing Surgery

Regina Elena Cancer Institute

TrialRECRUITING
Mar 2022Iconographic Registry of Pancreatobiliary Endoscopy Procedures

IRCCS San Raffaele

TrialRECRUITING
May 2021Spanish Series of Patients Treated With the Radionuclide Lutetium177

Sociedad Española de Medicina Nuclear e Imagen Molecular

TrialRECRUITING
May 2019Protocol ITANET - Registry

Italian Association Neuroendocrine Tumors

TrialRECRUITING

Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.

Treatments

No FDA-approved treatments are currently listed for Neuroendocrine carcinoma of pancreas.

1 clinical trialare actively recruiting — trials can provide access to cutting-edge therapies.

View clinical trials →

Clinical Trials

1 recruitingView all trials with filters →
Phase 21 trial
Efficacy and Safety Evaluation of Tarlatamab in Advanced Extrapulmonary Neuroendocrine Carcinoma Patients
Phase 2
Actively Recruiting
PI: Inkeun Park, M.D, Ph D (Asan Medical Center) · Sites: Seoul; Seoul +2 more · Age: 1999 yrs

Specialists

Showing 25 of 29View all specialists →
SM
Saleh K Saleh, MD
Minya, Minya Governorate
Specialist

Rare Disease Specialist

PI on 5 active trials
AP
Annamaria Colao, MD, PhD
Specialist
PI on 3 active trials
NM
Ning Zhong, MD
SEATTLE, WA
Specialist
PI on 2 active trials
MM
Matthew Kulke, MD
SOUTH PORTLAND, ME
Specialist
PI on 2 active trials
JM
Jonathan Strosberg, M.D.
TAMPA, FL
Specialist
PI on 6 active trials
JM
Jonathan Strosberg, MD
TAMPA, FL
Specialist
PI on 2 active trials
GM
Gianni Bisogno, MD
Specialist
PI on 2 active trials
LM
Lin Shen, MD
Specialist
PI on 8 active trials1 Neuroendocrine carcinoma of pancreas publication
LS
Lillian Siu
Edmonton, Alberta
Specialist

Rare Disease Specialist

PI on 4 active trials1 Neuroendocrine carcinoma of pancreas publication
JP
Julian R. Molina, M.D., Ph.D.
Specialist
PI on 1 active trial
RI
Renuka Iyer
BUFFALO, NY
Specialist
PI on 3 active trials33 Neuroendocrine carcinoma of pancreas publications
SM
Stergios Moschos, MD
Chapel Hill, North Carolina
Specialist

Rare Disease Specialist

PI on 3 active trials
AM
Atif Khan, MD
Specialist
PI on 1 active trial
MM
Manabu Kawai, MD
Specialist
PI on 2 active trials
CP
Carmina Valle, PhD
Specialist
PI on 1 active trial

Treatment Centers

8 centers
🏥 NORD

Baylor College of Medicine Rare Disease Center

Baylor College of Medicine

📍 Houston, TX

🏥 NORD

Stanford Medicine Rare Disease Center

Stanford Medicine

📍 Stanford, CA

🔬 UDN

NIH Clinical Center Undiagnosed Diseases Program

National Institutes of Health

📍 Bethesda, MD

🔬 UDN

UCLA UDN Clinical Site

UCLA Health

📍 Los Angeles, CA

🔬 UDN

Baylor College of Medicine UDN Clinical Site

Baylor College of Medicine

📍 Houston, TX

🔬 UDN

Harvard/MGH UDN Clinical Site

Massachusetts General Hospital

📍 Boston, MA

🏥 NORD

Mayo Clinic Center for Individualized Medicine

Mayo Clinic

📍 Rochester, MN

👤 Mayo Clinic Center for Individualized Medicine

🏥 NORD

UCLA Rare Disease Day Program

UCLA Health

📍 Los Angeles, CA

Travel Grants

No travel grants are currently matched to Neuroendocrine carcinoma of pancreas.

Search all travel grants →NORD Financial Assistance ↗

Community

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Latest news about Neuroendocrine carcinoma of pancreas

1 articles
Clinical trialUNITERAREApr 15, 2026
New Recruiting Trial: Temozolomide and Survivin Long Peptide Vaccine (SurVaxM) for the Treatment of Patients With Progressing Metastatic Neuroendocrine Carcinomas
Researchers are testing a new cancer treatment that combines two approaches: a chemotherapy drug called temozolomide and a vaccine called SurVaxM that trains th
See all news about Neuroendocrine carcinoma of pancreas

Caregiver Resources

NORD Caregiver Resources

Support, advocacy, and financial assistance for caregivers of rare disease patients.

Mental Health Support

Rare disease caregiving can be isolating. Connect with counseling and peer support.

Family & Caregiver Grants

Financial assistance programs specifically for caregivers of rare disease patients.

Social Security Disability

Learn how rare disease patients may qualify for SSDI/SSI benefits.

Questions for your doctor

Bring these to your next appointment

  • Q1.What stage is my cancer, and has it spread to other organs?,Is surgery an option for me, and what would that involve?,What chemotherapy regimen do you recommend, and what side effects should I expect?,Should I have genetic testing to see if a hereditary syndrome might be involved?,Are there any clinical trials I might be eligible for?,How will we know if the treatment is working, and how often will I be monitored?,What palliative care or supportive services are available to help manage my symptoms and quality of life?

Common questions about Neuroendocrine carcinoma of pancreas

What is Neuroendocrine carcinoma of pancreas?

Neuroendocrine carcinoma of the pancreas (also called pancreatic neuroendocrine carcinoma, or NEC of the pancreas) is a rare and aggressive type of cancer that starts in the hormone-producing cells of the pancreas. The pancreas has two main jobs: helping with digestion and making hormones like insulin that control blood sugar. Neuroendocrine carcinomas arise from the cells that make these hormones, but unlike the more common and slower-growing pancreatic neuroendocrine tumors (PNETs), neuroendocrine carcinomas grow and spread quickly. They are considered high-grade, meaning the cancer cells lo

How is Neuroendocrine carcinoma of pancreas inherited?

Neuroendocrine carcinoma of pancreas follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Neuroendocrine carcinoma of pancreas typically begin?

Typical onset of Neuroendocrine carcinoma of pancreas is adult. Age of onset can vary across affected individuals.

Are there clinical trials for Neuroendocrine carcinoma of pancreas?

Yes — 1 recruiting clinical trial is currently listed for Neuroendocrine carcinoma of pancreas on UniteRare. See the clinical trials section on this page for phase, sponsor, and site details sourced from ClinicalTrials.gov.

Which specialists treat Neuroendocrine carcinoma of pancreas?

25 specialists and care centers treating Neuroendocrine carcinoma of pancreas are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.