The first 60 days: Neuroendocrine tumours
A family of usually slow-growing tumours that start in hormone-producing cells of the gut, pancreas and lungs. They pioneered the idea of using the same molecule to see a tumour on a scan and then to treat it with radiation. Below, week by week, is what OnCo's record of 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.
- Diagnosis and stagingNCCN category Neuroendocrine and Adrenal Tumors, NCCN 2026 / ENETS 2023
Histology with Ki-67 grading; 68Ga/64Cu-DOTATATE PET/CT ± FDG PET; triple-phase CT or MRI of the liver; chromogranin A and syndrome-specific hormones; germline testing for pancreatic NETs and paragangliomas.
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, Neuroendocrine carcinoma (poorly differentiated).
- RadiologistNamed in the standard of care for: Diagnosis and staging.
- SurgeonNamed in the standard of care for: Localised, Localised disease, Advanced pancreatic NET needing tumour shrinkage, VHL-associated pancreatic NET.
- Medical oncologistNamed in the standard of care for: Advanced, Diagnosis and staging, Advanced, grade 1-2, SSTR-positive, first line, Advanced, progression on SSA and 5 more.
- Clinical oncologist (radiotherapy)Named in the standard of care for: Advanced, Advanced, grade 1-2, SSTR-positive, first line, Advanced, progression on SSA, Advanced pancreatic NET needing tumour shrinkage.
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.
Resection.
Surgical resection (including primary tumour resection with liver metastases where feasible); endoscopic resection for small rectal/gastric NETs; surveillance for small incidental lesions.
SSA → 177Lu-DOTATATE → everolimus/cabozantinib/chemotherapy; alpha therapy in trials.
- 4.Advanced, grade 1-2, SSTR-positive, first lineNCCN category Category 1 (SSA); category 1 PRRT for grade 2-3 first line
Somatostatin analogue (octreotide LAR or lanreotide); 177Lu-DOTATATE first line for grade 2-3 (NETTER-2) with high burden.
PRRT with 177Lu-DOTATATE (or 177Lu-edotreotide if approved); everolimus; sunitinib (pancreatic); cabozantinib (CABINET, all sites).
CAPTEM (E2211); PRRT; liver-directed therapy for hepatic-dominant disease.
SSA dose escalation; telotristat ethyl for refractory diarrhoea; octreotide infusion peri-procedurally; echocardiographic screening for carcinoid heart disease.
Platinum-etoposide (as in SCLC) ± PD-L1 inhibitor by extrapolation; FOLFIRINOX or CAPTEM in later lines; DLL3-directed agents in trials.
Everolimus or cabozantinib; alpha PRRT in trials (ACTION-1, AlphaMedix); PRRT retreatment in selected patients.
Belzutifan (approved 2021) for non-metastatic tumours not requiring immediate surgery.
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 grade, SSTR PET uptake, Chromogranin A, MEN1, DAXX/ATRX, Ki-67 index and mitotic count), 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 Small-bowelNET, often with carcinoid syndrome, Pancreatic NET, Lung NET.
- Is germline (inherited) genetic testing recommended for me or my family?Inherited variants can change treatment and matter for relatives.
Localised
- For my situation (localised), which of the standard options do you recommend and why?Guideline options include: Resection.
Advanced
- For my situation (advanced), which of the standard options do you recommend and why?Guideline options include: SSA → 177Lu-DOTATATE → everolimus/cabozantinib/chemotherapy; alpha therapy in trials.
- Am I a candidate for Lutetium-177 dotatate, Actinium-225 DOTATATE, and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.
Diagnosis and staging
- For my situation (diagnosis and staging), which of the standard options do you recommend and why?Guideline options include: Histology with Ki-67 grading; 68Ga/64Cu-DOTATATE PET/CT ± FDG PET; triple-phase CT or MRI of the liver; chromogranin A and syndrome-specific hormones; germline testing for pancreatic NETs and paragangliomas.
Localised disease
- For my situation (localised disease), which of the standard options do you recommend and why?Guideline options include: Surgical resection (including primary tumour resection with liver metastases where feasible); endoscopic resection for small rectal/gastric NETs; surveillance for small incidental lesions.
Advanced, grade 1-2, SSTR-positive, first line
- For my situation (advanced, grade 1-2, sstr-positive, first line), which of the standard options do you recommend and why?Guideline options include: Somatostatin analogue (octreotide LAR or lanreotide); 177Lu-DOTATATE first line for grade 2-3 (NETTER-2) with high burden.
- Am I a candidate for Somatostatin analogues (octreotide, lanreotide), Lutetium-177 dotatate, and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.
- How do the results of PROMID and CLARINET apply to someone like me?Trial populations differ from individual patients; ask how closely you match.
Advanced, progression on SSA
- For my situation (advanced, progression on ssa), which of the standard options do you recommend and why?Guideline options include: PRRT with 177Lu-DOTATATE (or 177Lu-edotreotide if approved); everolimus; sunitinib (pancreatic); cabozantinib (CABINET, all sites).
- Am I a candidate for Lutetium-177 dotatate, 177Lu-edotreotide, Everolimus 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 COMPETE and RADIANT-3 and RADIANT-4 apply to someone like me?Trial populations differ from individual patients; ask how closely you match.
Advanced pancreatic NET needing tumour shrinkage
- For my situation (advanced pancreatic net needing tumour shrinkage), which of the standard options do you recommend and why?Guideline options include: CAPTEM (E2211); PRRT; liver-directed therapy for hepatic-dominant disease.
- Am I a candidate for Capecitabine + temozolomide (CAPTEM), and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.
Carcinoid syndrome
- For my situation (carcinoid syndrome), which of the standard options do you recommend and why?Guideline options include: SSA dose escalation; telotristat ethyl for refractory diarrhoea; octreotide infusion peri-procedurally; echocardiographic screening for carcinoid heart disease.
- Am I a candidate for Somatostatin analogues (octreotide, lanreotide), and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.
Neuroendocrine carcinoma (poorly differentiated)
- For my situation (neuroendocrine carcinoma (poorly differentiated)), which of the standard options do you recommend and why?Guideline options include: Platinum-etoposide (as in SCLC) ± PD-L1 inhibitor by extrapolation; FOLFIRINOX or CAPTEM in later lines; DLL3-directed agents in trials.
- Am I a candidate for Carboplatin, Tarlatamab, Atezolizumab, and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.
After PRRT failure
- For my situation (after prrt failure), which of the standard options do you recommend and why?Guideline options include: Everolimus or cabozantinib; alpha PRRT in trials (ACTION-1, AlphaMedix); PRRT retreatment in selected patients.
- Am I a candidate for Actinium-225 DOTATATE, 212Pb-DOTAMTATE, and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.
- How do the results of ACTION-1 apply to someone like me?Trial populations differ from individual patients; ask how closely you match.
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 (approved 2021) for non-metastatic tumours not requiring immediate surgery.
- 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.
Any stage
- Are there clinical trials I could join, for example of Actinium-225 DOTATATE, PM8002, ZG006, Zanzalintinib?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 “Neuroendocrine carcinoma (high grade) behaves like SCLC”. How does that affect my plan?Open problems are where trials and second opinions matter most.
- I read that “Sequencing of PRRT vs targeted therapy”. 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
- Carcinoid Syndrome Efficacy Study Featuring an Oral Daily Paltusotine RegimenPhase 3 · recruiting · NCT07087054A Randomized, Parallel Group, Placebo-Controlled Study to Evaluate the Efficacy and Safety of Paltusotine in Adults With Carcinoid Syndrome Due to Well-Differentiated Neuroendocrine Tumors
- DAREON®-NEC-1: A Study in People With Advanced Extrapulmonary Neuroendocrine Cancer (epNEC) to Compare Obrixtamig Plus Carboplatin and Etoposide Treatment With Standard ChemotherapyPhase 3 · recruiting · NCT07544654A Phase III, Multi-center, Open-label, Randomised, Controlled Trial of Intravenous Obrixtamig in Combination With Carboplatin and Etoposide vs. Carboplatin and Etoposide as First-line Therapy in DLL3-positive Patients With Unresectable Locally Advanced or Metastatic Extrapulmonary Neuroendocrine Carcinomas
Much of what helps in the first weeks is free if you know to ask: testing, helplines, rides and lodging, second opinions, trial travel.
- Neuroendocrine tumours: the full pageA family of usually slow-growing tumours that start in hormone-producing cells of the gut, pancreas and lungs. They pioneered the idea of using the same molecule to see a tumour on a scan and then to treat it with radiation.
- 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.
- Alpha vs beta emitters: Beta particles (lutetium-177) travel millimetres and are good for bulky disease; alpha particles (actinium-225) travel a few cells' width and kill with far higher energy.
- 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.
- Carcinoid syndrome and carcinoid heart disease: Flushing, diarrhoea and wheezing caused by hormones (mostly serotonin) released by some neuroendocrine tumours; over years it can scar the heart valves.
- 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.
- Dosimetry: Measuring how much radiation dose each organ and tumour actually received from a radioactive drug.
- 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.
- TACE (transarterial chemoembolisation): Threading a catheter into the artery feeding a liver tumour and injecting chemotherapy plus particles that block the blood supply, starving and poisoning it at once.
- Von Hippel-Lindau disease: Von Hippel-Lindau disease is an inherited condition causing kidney cancers, adrenal tumours, and blood-vessel tumours of the brain, spine, eye and pancreas from early adulthood.
- 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.
- Theranostics: Using the same targeting molecule for a diagnostic scan and a therapy, so you treat exactly what you can see.
Every term links to the glossary.