The first 60 days: Small intestinal neuroendocrine tumours

Staging tests establish exactly what and where the cancer is. Everything else follows from the answers.

• Diagnosis and stagingNCCN Guidelines: Neuroendocrine and Adrenal Tumors

  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.

  Somatostatin receptor PET (68Ga/64Cu-DOTATATE)Gallium-68 DOTATATE (and Cu-64 DOTATATE)Neuroendocrine tumour grade (Ki-67) and WHO classificationChromogranin ACarcinoid syndrome and carcinoid heart disease

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.

1. 1.Localised or resectable diseaseNCCN Guidelines: Neuroendocrine and Adrenal Tumors

   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.

   Robotic & minimally invasive surgeryHepatectomy (liver resection)
2. 2.Advanced, first lineNCCN Guidelines: Neuroendocrine and Adrenal Tumors

   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).

   Somatostatin analogues (octreotide, lanreotide)PROMIDCLARINETLutetium-177 dotatateStudy to Evaluate the Efficacy and Safety of Lutathera in Patients With Grade 2 and Grade 3 Advanced GEP-NETPeptide receptor radionuclide therapy (PRRT)
3. 3.Progression on a somatostatin analogueNCCN Guidelines: Neuroendocrine and Adrenal Tumors

   Lutetium-177 dotatate (NETTER-1); everolimus (RADIANT-4); cabozantinib (CABINET); liver-directed therapy for hepatic-dominant disease.

   Lutetium-177 dotatateNETTER-1EverolimusRADIANT-3 and RADIANT-4CabozantinibCABINET (Alliance A021602)Transarterial chemoembolisation (TACE)Radioembolisation (TARE / SIRT, yttrium-90)Thermal ablation (RFA, microwave, cryo)Liver-directed therapy (TACE, TARE, HAI, ablation)
4. 4.Carcinoid syndromeNCCN Guidelines: Neuroendocrine and Adrenal Tumors

   Somatostatin analogue dose escalation, telotristat ethyl for refractory diarrhoea, octreotide infusion around procedures to prevent carcinoid crisis, valve surgery for carcinoid heart disease.

   Somatostatin analogues (octreotide, lanreotide)Telotristat ethylCarcinoid syndrome and carcinoid heart disease
5. 5.After radioligand therapyNCCN Guidelines: Neuroendocrine and Adrenal Tumors

   Everolimus or cabozantinib; 177Lu-edotreotide if approved; alpha-emitting radioligands and retreatment in trials.

   EverolimusCabozantinib177Lu-edotreotideCOMPETEActinium-225 DOTATATEACTION-1212Pb-DOTAMTATE

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.

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.

• Free in oncologyFree testing, helplines, rides and lodging, second opinions and trial travel help, with who is eligible.
• Financial helpManufacturer programmes, national schemes and charities by country and product.
• Second opinionExpert centres for this cancer in your country and how referral works.

• 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.