The first 60 days: Pancreatic neuroendocrine tumours
Pancreatic neuroendocrine tumours arise from the hormone-producing islet cells of the pancreas and behave very differently from ordinary pancreatic cancer, often growing for years. Surgery cures localised tumours; advanced disease is treated in sequence with somatostatin analogues, lutetium-177 dotatate, targeted tablets and oral chemotherapy, and a minority secrete insulin or gastrin. Below, week by week, is what OnCo's record of Pancreatic neuroendocrine tumours says about the first two months: the order is typical, the timing is yours to ask about. Sections appear only where the record has something to say. Orientation, not medical advice.
What happens now
Staging tests establish exactly what and where the cancer is. Everything else follows from the answers.
Contrast CT or MRI, somatostatin receptor PET, biopsy with Ki-67 grading, chromogranin A, hormone assays where a syndrome is suspected, and germline testing.
Ask which of these were tested and what the results were; see the report reader for what each value means.
These specialties appear in the standard of care for this cancer. In most centres they meet weekly as a tumour board to agree each plan; you can ask when yours was discussed and what was decided.
- PathologistNamed in the standard of care for: Diagnosis and staging, Liver-dominant disease.
- RadiologistNamed in the standard of care for: Diagnosis and staging.
- SurgeonNamed in the standard of care for: Localised, resectable, Functioning syndromes, Liver-dominant disease, VHL-associated pancreatic NET.
- Medical oncologistNamed in the standard of care for: Diagnosis and staging, Functioning syndromes, Advanced, first line, Progression on a somatostatin analogue and 2 more.
- Clinical oncologist (radiotherapy)Named in the standard of care for: Diagnosis and staging, Functioning syndromes, Advanced, first line, Progression on a somatostatin analogue and 1 more.
A second opinion from a centre that treats many similar cases is normal, not rude; the standard of care tells you what to compare it against.
Each row is a setting from the standard of care, in the order it usually arises. Not all will apply to you; your stage and biomarkers decide which do. The guideline grade, where recorded, says how strong the evidence is.
Enucleation or distal pancreatectomy for small tumours, Whipple procedure for tumours in the head, lymphadenectomy for tumours over two centimetres; surveillance for small non-functioning tumours.
Surgery for insulinoma with diazoxide or everolimus to control hypoglycaemia beforehand; high-dose proton-pump inhibitors and resection for gastrinoma; somatostatin analogues for glucagonoma and VIPoma.
Lanreotide or octreotide (CLARINET); lutetium-177 dotatate first line for grade 2 to 3 tumours with a Ki-67 of 10 percent or more (NETTER-2); CAPTEM when shrinkage is needed.
Lutetium-177 dotatate; everolimus (RADIANT-3); sunitinib; cabozantinib (CABINET); CAPTEM or streptozocin-based chemotherapy.
Resection, thermal ablation, chemoembolisation or radioembolisation, alongside systemic therapy.
Belzutifan for tumours not requiring immediate surgery (approved 2021).
Generated from this cancer's standard of care, biomarkers and open problems. Take the group that matches where you are. To tick, add your own and print, open the one-page appointment sheet.
Newly diagnosed
- What is my exact diagnosis, stage, and grade, and which tests established them?Everything else follows from an accurate stage and subtype.
- Which biomarkers have been tested on my tumour (for example Ki-67 index and mitotic count, Chromogranin A, Somatostatin receptor PET, Fasting glucose, insulin, C-peptide and proinsulin, Fasting gastrin and gastric pH), and what were the results?These results decide eligibility for targeted therapy, immunotherapy, and trials.
- Which subtype is my cancer, and does that change the recommended treatment?Recognised subtypes for this cancer include Non-functioning pancreatic NET, Insulinoma, Gastrinoma and Zollinger-Ellison syndrome.
- Is germline (inherited) genetic testing recommended for me or my family?Inherited variants can change treatment and matter for relatives.
Diagnosis and staging
- For my situation (diagnosis and staging), which of the standard options do you recommend and why?Guideline options include: Contrast CT or MRI, somatostatin receptor PET, biopsy with Ki-67 grading, chromogranin A, hormone assays where a syndrome is suspected, and germline testing.
- Am I a candidate for Gallium-68 DOTATATE (and Cu-64 DOTATATE), and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.
Localised, resectable
- For my situation (localised, resectable), which of the standard options do you recommend and why?Guideline options include: Enucleation or distal pancreatectomy for small tumours, Whipple procedure for tumours in the head, lymphadenectomy for tumours over two centimetres; surveillance for small non-functioning tumours.
Functioning syndromes
- For my situation (functioning syndromes), which of the standard options do you recommend and why?Guideline options include: Surgery for insulinoma with diazoxide or everolimus to control hypoglycaemia beforehand; high-dose proton-pump inhibitors and resection for gastrinoma; somatostatin analogues for glucagonoma and VIPoma.
- Am I a candidate for Somatostatin analogues (octreotide, lanreotide), Everolimus, and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.
Advanced, first line
- For my situation (advanced, first line), which of the standard options do you recommend and why?Guideline options include: Lanreotide or octreotide (CLARINET); lutetium-177 dotatate first line for grade 2 to 3 tumours with a Ki-67 of 10 percent or more (NETTER-2); CAPTEM when shrinkage is needed.
- Am I a candidate for Somatostatin analogues (octreotide, lanreotide), Lutetium-177 dotatate, Capecitabine + temozolomide (CAPTEM), and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.
- How do the results of CLARINET and Study to Evaluate the Efficacy and Safety of Lutathera in Patients With Grade 2 and Grade 3 Advanced GEP-NET apply to someone like me?Trial populations differ from individual patients; ask how closely you match.
Progression on a somatostatin analogue
- For my situation (progression on a somatostatin analogue), which of the standard options do you recommend and why?Guideline options include: Lutetium-177 dotatate; everolimus (RADIANT-3); sunitinib; cabozantinib (CABINET); CAPTEM or streptozocin-based chemotherapy.
- Am I a candidate for Lutetium-177 dotatate, Everolimus, Sunitinib or related drugs, and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.
- How do the results of RADIANT-3 and RADIANT-4 and CABINET (Alliance A021602) apply to someone like me?Trial populations differ from individual patients; ask how closely you match.
Liver-dominant disease
- For my situation (liver-dominant disease), which of the standard options do you recommend and why?Guideline options include: Resection, thermal ablation, chemoembolisation or radioembolisation, alongside systemic therapy.
VHL-associated pancreatic NET
- For my situation (vhl-associated pancreatic net), which of the standard options do you recommend and why?Guideline options include: Belzutifan for tumours not requiring immediate surgery (approved 2021).
- Am I a candidate for Belzutifan, and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.
- How do the results of Belzutifan/MK-6482 for the Treatment of Advanced Pheochromocytoma/Paraganglioma (PPGL), Pancreatic Neuroendocrine Tumor (pNET), Von Hippel-Lindau (VHL apply to someone like me?Trial populations differ from individual patients; ask how closely you match.
Any stage
- Are there clinical trials I could join, for example of 177Lu-edotreotide, COMPETE, Lutetium 177Lu-Edotreotide Versus Best Standard of Care in Well-differentiated Aggressive Grade-2 and Grade-3 GastroEnteroPancreatic NeuroEndocrine Tumors (GEP-NETs) - COMPOSE, Belzutifan/MK-6482 for the Treatment of Advanced Pheochromocytoma/Paraganglioma (PPGL), Pancreatic Neuroendocrine Tumor (pNET), Von Hippel-Lindau (VHL?Trials are how the next standard of care is set; asking early keeps options open.
- Would a second opinion at a high-volume centre change anything, and can you help arrange it?Rare or high-stakes decisions benefit from a centre that treats many similar patients.
- What supportive care (symptom control, nutrition, exercise, mental health, financial help) is available from the start?Supportive care improves quality of life and helps patients complete treatment.
- I read that “The best order of radioligand therapy, everolimus, sunitinib, cabozantinib and CAPTEM is unknown”. How does that affect my plan?Open problems are where trials and second opinions matter most.
- I read that “Which small non-functioning tumours can safely be watched rather than resected”. How does that affect my plan?Open problems are where trials and second opinions matter most.
Trials open now for this cancer in OnCo, largest phase first. Joining a trial is a decision like any other: ask what the comparison arm is, whether a placebo is used, and what happens if you leave. The cancer page searches ClinicalTrials.gov live for more.
- A Study Comparing Treatment With Lutetium[177Lu] Oxodotreotide Injection to Octreotide LAR in Patients With GEP-NETsPhase 3 · active · NCT05459844A Study Comparing Treatment With Lutetium[177Lu] Oxodotreotide Injection to Octreotide LAR in Patients With Inoperable, Progressive, Well Differentiated, Somatostatin Receptor Positive Gastroenteropancreatic Neuroendocrine Tumours
- A Trial to Assess Efficacy and Safety of Octreotide Subcutaneous Depot in Patients With GEP-NETPhase 3 · active · NCT05050942A Randomized, Multi-center, Open-label, Active-controlled Phase 3 Trial to Assess the Efficacy and Safety of Octreotide Subcutaneous Depot (CAM2029) Versus Octreotide LAR or Lanreotide ATG in Patients With GEP-NET
- ACTION-1Phase 3 · recruiting · NCT05477576SSTR-positive GEP-NETs progressing after 177Lu somatostatin-analogue therapy: 225Ac-DOTATATE (RYZ101) vs investigator's choice
- An Open-label Phase 3 Study of Lutetium (177Lu) Oxodotreotide Injection in Subjects With Advanced Gastrointestinal Pancreatic Neuroendocrine Tumors.Phase 3 · recruiting · NCT05884255Randomized, Open, Positive Control Phase III Clinical Trial of Lutetium (177Lu) Oxodotreotide Injection Combined With Standard-dose Long-acting Octreotide Versus High-dose Long-acting Octreotide in the Treatment of Somatostatin Receptor-positive Advanced Gastrointestinal Pancreatic Neuroendocrine Tumors.
- Lutetium 177Lu-Edotreotide Versus Best Standard of Care in Well-differentiated Aggressive Grade-2 and Grade-3 GastroEnteroPancreatic NeuroEndocrine Tumors (GEP-NETs) - COMPOSEPhase 3 · active · NCT04919226A Prospective, Randomised, Controlled, Open-label, Multicentre Study to Evaluate Efficacy, Safety and Patient-Reported Outcomes of Peptide Receptor Radionuclide Therapy (PRRT) With 177Lu-Edotreotide Compared to Best Standard of Care in Patients With Well-differentiated Aggressive Grade 2 and Grade 3, Somatostatin Receptor-Positive (SSTR+), Neuroendocrine Tumours of GastroEnteric or Pancreatic Origin
Much of what helps in the first weeks is free if you know to ask: testing, helplines, rides and lodging, second opinions, trial travel.
- Pancreatic neuroendocrine tumours: the full pagePancreatic neuroendocrine tumours arise from the hormone-producing islet cells of the pancreas and behave very differently from ordinary pancreatic cancer, often growing for years. Surgery cures localised tumours; advanced disease is treated in sequence with somatostatin analogues, lutetium-177 dotatate, targeted tablets and oral chemotherapy, and a minority secrete insulin or gastrin.
- One-page appointment sheetYour questions, the words you may hear, what to bring, and space for the answers. Print it.
- Treatment sequencingWhich treatment tends to follow which, line by line.
- NavigatorStandard of care for your stage, what you have tried, and trials near you.
- MEN1 and hereditary neuroendocrine syndromes: Inherited conditions (MEN1, VHL, NF1, tuberous sclerosis) that cause neuroendocrine tumours, often multiple and at a young age, so families need genetic testing and surveillance.
- PRRT (peptide receptor radionuclide therapy): A radioactive drug for neuroendocrine tumours: a small peptide that homes to the somatostatin receptor on the tumour cells carries lutetium-177, which irradiates them from within.
- Chromogranin A: Chromogranin A is a protein released by neuroendocrine cells and measured in blood to follow tumour burden; it is unreliable because acid-reducing drugs and kidney disease also raise it.
- Whipple procedure (pancreaticoduodenectomy): The big operation for cancers of the head of the pancreas: the surgeon removes the pancreatic head, the duodenum, the gallbladder and part of the bile duct, then reconnects everything.
- Neuroendocrine tumour grade (Ki-67) and WHO classification: How fast the tumour cells are dividing, measured by Ki-67 staining, separates slow-growing neuroendocrine tumours from aggressive neuroendocrine carcinomas and decides the treatment.
- Liver-directed therapy (TACE, TARE, HAI, ablation): The set of treatments aimed only at tumours in the liver, delivered through its artery or by needle, used when the liver is the main or only site of disease: chemoembolisation, radioactive beads, ablation and infusion pumps.
- Hepatectomy (liver resection): Cutting out the part of the liver containing tumour.
Every term links to the glossary.