The first 60 days: Small intestinal neuroendocrine tumours
Small intestinal neuroendocrine tumours are slow-growing hormone-producing tumours of the ileum and jejunum, often found only after they have spread to lymph nodes and the liver. Monthly somatostatin analogue injections control symptoms and growth, lutetium-177 dotatate is the main second treatment, and everolimus, cabozantinib and surgery fill in. Below, week by week, is what OnCo's record of Small intestinal 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.
Biopsy with Ki-67 grading, somatostatin receptor PET, cross-sectional imaging of the liver, chromogranin A and urinary 5-HIAA, echocardiography if carcinoid syndrome is present.
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.
- RadiologistNamed in the standard of care for: Diagnosis and staging.
- SurgeonNamed in the standard of care for: Localised or resectable disease, Progression on a somatostatin analogue, Carcinoid syndrome.
- Medical oncologistNamed in the standard of care for: Diagnosis and staging, Advanced, first line, Progression on a somatostatin analogue, Carcinoid syndrome and 1 more.
- Clinical oncologist (radiotherapy)Named in the standard of care for: Diagnosis and staging, Advanced, first line, Progression on a somatostatin analogue, After radioligand therapy.
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.
Segmental small bowel resection with mesenteric lymphadenectomy, inspecting the whole small bowel for further primaries; the primary is often removed even when liver metastases are present.
Octreotide LAR or lanreotide (PROMID, CLARINET); lutetium-177 dotatate first line for grade 2 to 3 tumours with a Ki-67 of 10 percent or more (NETTER-2).
Lutetium-177 dotatate (NETTER-1); everolimus (RADIANT-4); cabozantinib (CABINET); liver-directed therapy for hepatic-dominant disease.
Somatostatin analogue dose escalation, telotristat ethyl for refractory diarrhoea, octreotide infusion around procedures to prevent carcinoid crisis, valve surgery for carcinoid heart disease.
Everolimus or cabozantinib; 177Lu-edotreotide if approved; alpha-emitting radioligands and retreatment in trials.
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, 24-hour urinary 5-HIAA, Somatostatin receptor PET with gallium-68 or copper-64 DOTATATE, Echocardiography for carcinoid heart disease), 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 Ileal neuroendocrine tumour, Jejunal neuroendocrine tumour, Duodenal neuroendocrine tumour.
- 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: Biopsy with Ki-67 grading, somatostatin receptor PET, cross-sectional imaging of the liver, chromogranin A and urinary 5-HIAA, echocardiography if carcinoid syndrome is present.
- 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 or resectable disease
- For my situation (localised or resectable disease), which of the standard options do you recommend and why?Guideline options include: Segmental small bowel resection with mesenteric lymphadenectomy, inspecting the whole small bowel for further primaries; the primary is often removed even when liver metastases are present.
Advanced, first line
- For my situation (advanced, first line), which of the standard options do you recommend and why?Guideline options include: Octreotide LAR or lanreotide (PROMID, CLARINET); lutetium-177 dotatate first line for grade 2 to 3 tumours with a Ki-67 of 10 percent or more (NETTER-2).
- 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.
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 (NETTER-1); everolimus (RADIANT-4); cabozantinib (CABINET); liver-directed therapy for hepatic-dominant disease.
- Am I a candidate for Lutetium-177 dotatate, Everolimus, Cabozantinib, and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.
- How do the results of NETTER-1 and RADIANT-3 and RADIANT-4 apply to someone like me?Trial populations differ from individual patients; ask how closely you match.
Carcinoid syndrome
- For my situation (carcinoid syndrome), which of the standard options do you recommend and why?Guideline options include: Somatostatin analogue dose escalation, telotristat ethyl for refractory diarrhoea, octreotide infusion around procedures to prevent carcinoid crisis, valve surgery for carcinoid heart disease.
- Am I a candidate for Somatostatin analogues (octreotide, lanreotide), Telotristat ethyl, and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.
After radioligand therapy
- For my situation (after radioligand therapy), which of the standard options do you recommend and why?Guideline options include: Everolimus or cabozantinib; 177Lu-edotreotide if approved; alpha-emitting radioligands and retreatment in trials.
- Am I a candidate for Everolimus, Cabozantinib, 177Lu-edotreotide 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 ACTION-1 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, Actinium-225 DOTATATE, ACTION-1?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 “No randomised trial orders radioligand therapy, everolimus and cabozantinib after somatostatin analogues”. How does that affect my plan?Open problems are where trials and second opinions matter most.
- I read that “Whether removing the primary improves survival in patients with liver metastases has never been tested prospectively”. 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
- 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.
- Small intestinal neuroendocrine tumours: the full pageSmall intestinal neuroendocrine tumours are slow-growing hormone-producing tumours of the ileum and jejunum, often found only after they have spread to lymph nodes and the liver. Monthly somatostatin analogue injections control symptoms and growth, lutetium-177 dotatate is the main second treatment, and everolimus, cabozantinib and surgery fill in.
- 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.
- 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.
- 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.
- 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.