Pancreatic neuroendocrine tumours: the decisions you may face

Cancer page →Second-opinion finder →

7 treatment settings, 5 with more than one named option. Each section lays out the options the standard of care names, what each is for, the trials behind them with their recorded results, the side effects and cautions on record, and questions to ask. Built from the cancer page's standard-of-care rows; nothing here is advice for your case.

Screening, prevention and diagnosis

Diagnosis and staging

3 options

Contrast CT or MRI, somatostatin receptor PET, biopsy with Ki-67 grading, chromogranin A, hormone assays where a syndrome is suspected, and germline testing.

Guideline

NCCN Guidelines: Neuroendocrine and Adrenal Tumors

The options, in plain words

Somatostatin receptor PET (68Ga/64Cu-DOTATATE)Standard of care

A PET scan using a radioactive hormone mimic that lights up neuroendocrine tumours and shows whether the matching radioactive treatment will work.

Whole-body receptor map
Theranostic gatekeeper for 177Lu-DOTATATE
Changes management in ~40% of patients versus conventional imaging

Gallium-68 DOTATATE (and Cu-64 DOTATATE)ApprovedPET radiotracer (somatostatin receptor ligand)

The PET scan for neuroendocrine tumours that finds far more disease than older scans and confirms eligibility for lutetium radioligand therapy (the theranostic pair).

Germline (hereditary) testingStandard of care

A test of the DNA you were born with, to find inherited risk genes such as BRCA or Lynch syndrome.

Actionable for patient and relatives
Cheap

Also referenced:Neuroendocrine tumour grade (Ki-67) and WHO classificationChromogranin AMEN1 and hereditary neuroendocrine syndromes

The evidence behind it

No trial record is attached to this row yet. The trials tab lists what is recruiting and the landmark trials for this cancer.

The main trade-offs on record

Somatostatin receptor PET (68Ga/64Cu-DOTATATE)

Physiologic uptake in pancreas uncinate, spleen, pituitary
Poor sensitivity in SSTR-negative high-grade disease
68Ga generator supply and short half-life

Germline (hereditary) testing

VUS burden
Uptake and counselling capacity

Questions to ask about this decision

Between Somatostatin receptor PET (68Ga/64Cu-DOTATATE), Gallium-68 DOTATATE (and Cu-64 DOTATATE) and Germline (hereditary) testing, which do you recommend for me, and what about my case would make you choose differently?
Why: Guidelines list several reasonable options; the choice turns on details of your tumour, your health and your priorities.
What is each option trying to achieve: cure, long control, or relief of symptoms, and over what time?
Why: The aim shapes how much side effect and disruption is worth accepting.
What happens if I delay, or decline this step for now? Is the decision reversible?
Why: Some decisions can wait for a second opinion or a trial slot; others cannot. Knowing which is part of the choice.
Does your recommendation follow the current guideline (NCCN Guidelines: Neuroendocrine and Adrenal Tumors), and if it departs from it, why?
Why: Departures from guidelines are sometimes right for an individual; they should be explained.
For my situation (diagnosis and staging), which of the standard options do you recommend and why?
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?
Why: Knowing the expected toxicities helps you plan work, family, and supportive care.

Add these to your appointment list, or take the full question set for this cancer.

Early / localised

Localised, resectable

One path named

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.

Guideline

NCCN Guidelines: Neuroendocrine and Adrenal Tumors

The path, in plain words

Robotic & minimally invasive surgeryStandard of care

Surgeons operate through small incisions using robotic arms with tremor-free precision and 3D vision.

Precision, shorter stay
Enables complex minimally invasive resections

Also referenced:Whipple procedure (pancreaticoduodenectomy)

The evidence behind it

No trial record is attached to this row yet. The trials tab lists what is recruiting and the landmark trials for this cancer.

The main trade-offs on record

Robotic & minimally invasive surgery

Cost
Loss of haptic feedback
Not superior for every indication

Questions to ask about this decision

Is Robotic & minimally invasive surgery the only reasonable path for me, or is there a trial, a different sequence or a wait-and-see option?
Why: A single standard does not mean a single choice; timing and trials are decisions too.
What is each option trying to achieve: cure, long control, or relief of symptoms, and over what time?
Why: The aim shapes how much side effect and disruption is worth accepting.
What happens if I delay, or decline this step for now? Is the decision reversible?
Why: Some decisions can wait for a second opinion or a trial slot; others cannot. Knowing which is part of the choice.
Does your recommendation follow the current guideline (NCCN Guidelines: Neuroendocrine and Adrenal Tumors), and if it departs from it, why?
Why: Departures from guidelines are sometimes right for an individual; they should be explained.
For my situation (localised, resectable), which of the standard options do you recommend and why?
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.

Add these to your appointment list, or take the full question set for this cancer.

Other settings

Functioning syndromes

2 optionsCompare side by side →

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.

Guideline

NCCN Guidelines: Neuroendocrine and Adrenal Tumors

The options, in plain words

Somatostatin analogues (octreotide, lanreotide)ApprovedPeptide hormone analogue (SSTR2 agonist)

Monthly injections of a synthetic hormone that both quiets tumour hormone symptoms and slows tumour growth, the first treatment for most neuroendocrine tumours.

EverolimusApprovedSmall-molecule mTOR inhibitor

An mTOR-blocking pill that doubled progression-free time with exemestane in 2012 and is now paired with the oral SERD giredestrant.

Also referenced:MEN1 and hereditary neuroendocrine syndromes

The evidence behind it

No trial record is attached to this row yet. The trials tab lists what is recruiting and the landmark trials for this cancer.

The main trade-offs on record

Everolimus all recorded rates →

Avoid grapefruit. Live vaccines are contraindicated.
7.5 mg (mild), 5 mg (moderate), 2.5 mg (severe).

Questions to ask about this decision

Between Somatostatin analogues (octreotide, lanreotide) and Everolimus, which do you recommend for me, and what about my case would make you choose differently?
Why: Guidelines list several reasonable options; the choice turns on details of your tumour, your health and your priorities.
What is each option trying to achieve: cure, long control, or relief of symptoms, and over what time?
Why: The aim shapes how much side effect and disruption is worth accepting.
What happens if I delay, or decline this step for now? Is the decision reversible?
Why: Some decisions can wait for a second opinion or a trial slot; others cannot. Knowing which is part of the choice.
Does your recommendation follow the current guideline (NCCN Guidelines: Neuroendocrine and Adrenal Tumors), and if it departs from it, why?
Why: Departures from guidelines are sometimes right for an individual; they should be explained.
For my situation (functioning syndromes), which of the standard options do you recommend and why?
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?
Why: Knowing the expected toxicities helps you plan work, family, and supportive care.

Add these to your appointment list, or take the full question set for this cancer.

Advanced, first line

4 optionsCompare side by side →

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.

Guideline

NCCN Guidelines: Neuroendocrine and Adrenal Tumors

The options, in plain words

Somatostatin analogues (octreotide, lanreotide)ApprovedPeptide hormone analogue (SSTR2 agonist)

Monthly injections of a synthetic hormone that both quiets tumour hormone symptoms and slows tumour growth, the first treatment for most neuroendocrine tumours.

Lutetium-177 dotatateApprovedRadioligand therapy (beta)

Lutetium-177 dotatate was the first modern radioligand therapy (2018), for neuroendocrine tumours, and is now used in first line.

Peptide receptor radionuclide therapy (PRRT)Approved

A radioactive version of the hormone mimic used for the scan; it homes to neuroendocrine tumour cells and irradiates them from inside.

Systemic, receptor-targeted
Response and quality-of-life benefit
Imaging selects and monitors

Capecitabine + temozolomide (CAPTEM)EstablishedOral cytotoxic regimen

CAPTEM (capecitabine plus temozolomide) is an all-oral chemotherapy pair that shrinks pancreatic neuroendocrine tumours in about a third of patients.

The evidence behind it

CLARINET Phase 3 NCT00353496 2014204 peopleevidence 76
Tests Somatostatin analogues (octreotide, lanreotide)
Non-functioning enteropancreatic NETs, grade 1-2: lanreotide 120 mg vs placebo
PFS HR 0.47.
Progression-free survival (months): Placebo 18 (n=103) · HR 0.47 · source
Study to Evaluate the Efficacy and Safety of Lutathera in Patients With Grade 2 and Grade 3 Advanced GEP-NET Phase 3 NCT03972488226 peopleevidence 47
Tests Lutetium-177 dotatate
A Phase III Multi-center, Randomized, Open-label Study to Evaluate the Efficacy and Safety of Lutathera in Patients With Grade 2 and Grade 3 Advanced GEP-NET
No headline result recorded yet.

The main trade-offs on record

Lutetium-177 dotatate all recorded rates →

Side effect	Any grade	Grade 3+
Lymphopenia · NETTER-1; grade 3-4 rates	-	44%
GGT increased · NETTER-1; grade 3-4 rates	-	20%
Vomiting · NETTER-1; grade 3-4 rates	-	7%
Nausea · NETTER-1; grade 3-4 rates	-	5%

Rates from the label or pivotal trial as recorded on the product page; each grade 3+ figure links to its source.

Peptide receptor radionuclide therapy (PRRT)

Myelosuppression, rare MDS/AML (~2-3%)
Renal dose
Not curative; retreatment data limited

Questions to ask about this decision

Between Somatostatin analogues (octreotide, lanreotide), Lutetium-177 dotatate, Peptide receptor radionuclide therapy (PRRT) and the other options, which do you recommend for me, and what about my case would make you choose differently?
Why: Guidelines list several reasonable options; the choice turns on details of your tumour, your health and your priorities.
What is each option trying to achieve: cure, long control, or relief of symptoms, and over what time?
Why: The aim shapes how much side effect and disruption is worth accepting.
How closely do I match the people in CLARINET and Study to Evaluate the Efficacy and Safety of Lutathera in Patients With Grade 2 and Grade 3 Advanced GEP-NET, and does that change what the results mean for me?
Why: Trial populations are selected; age, fitness, prior treatment and biomarkers all affect how far results carry.
Which side effects of Lutetium-177 dotatate are most likely for me, which are reversible, and which would make us stop?
Why: The recorded rates below come from labels and trials; your own risk depends on dose, other medicines and your health.
What happens if I delay, or decline this step for now? Is the decision reversible?
Why: Some decisions can wait for a second opinion or a trial slot; others cannot. Knowing which is part of the choice.
Does your recommendation follow the current guideline (NCCN Guidelines: Neuroendocrine and Adrenal Tumors), and if it departs from it, why?
Why: Departures from guidelines are sometimes right for an individual; they should be explained.
For my situation (advanced, first line), which of the standard options do you recommend and why?
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?
Why: 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?
Why: Trial populations differ from individual patients; ask how closely you match.

Add these to your appointment list, or take the full question set for this cancer.

Second line

Progression on a somatostatin analogue

6 optionsCompare side by side →

Lutetium-177 dotatate; everolimus (RADIANT-3); sunitinib; cabozantinib (CABINET); CAPTEM or streptozocin-based chemotherapy.

Guideline

NCCN Guidelines: Neuroendocrine and Adrenal Tumors

The options, in plain words

Lutetium-177 dotatateApprovedRadioligand therapy (beta)

Lutetium-177 dotatate was the first modern radioligand therapy (2018), for neuroendocrine tumours, and is now used in first line.

EverolimusApprovedSmall-molecule mTOR inhibitor

An mTOR-blocking pill that doubled progression-free time with exemestane in 2012 and is now paired with the oral SERD giredestrant.

SunitinibApprovedSmall-molecule multi-kinase inhibitor (VEGFR, PDGFR, KIT)

Sunitinib is an anti-angiogenic pill approved for pancreatic neuroendocrine tumours, kidney cancer and GIST.

CabozantinibApprovedSmall-molecule multi-kinase inhibitor (MET, VEGFR2, AXL, RET)

A pill that blocks both blood-vessel growth and the MET escape pathway, used in kidney, liver, thyroid and, since 2025, neuroendocrine cancers.

Capecitabine + temozolomide (CAPTEM)EstablishedOral cytotoxic regimen

CAPTEM (capecitabine plus temozolomide) is an all-oral chemotherapy pair that shrinks pancreatic neuroendocrine tumours in about a third of patients.

StreptozocinEstablishedNitrosourea cytotoxic with glucose moiety, intravenous

Streptozocin is a chemotherapy drug that seeks out pancreatic islet cells, approved in 1982 for the rare tumours that arise from them; the same selectivity makes it the standard way to induce diabetes in laboratory animals.

The evidence behind it

RADIANT-3 and RADIANT-4 Phase 3 NCT00510068 2011712 peopleevidence 78
Tests Everolimus
Progressive pancreatic NETs (RADIANT-3, n=410) and lung/GI NETs (RADIANT-4, n=302): everolimus vs placebo
PFS HR 0.35 (pNET) and 0.48 (lung/GI).
Progression-free survival (RADIANT-3) (months): Everolimus 11 (n=207) vs Placebo 4.6 (n=203) · HR 0.35 · source
CABINET (Alliance A021602)Phase 3 NCT03375320 2024298 peopleevidence 76
Tests Cabozantinib
Previously treated advanced pancreatic (n=95) and extra-pancreatic (n=203) NETs: cabozantinib vs placebo
PFS HR 0.23 (pNET), 0.38 (epNET).
Progression-free survival (pancreatic NET) (months): Cabozantinib 13.8 vs Placebo 4.4 · HR 0.23 · source

The main trade-offs on record

Lutetium-177 dotatate all recorded rates →

Side effect	Any grade	Grade 3+
Lymphopenia · NETTER-1; grade 3-4 rates	-	44%
GGT increased · NETTER-1; grade 3-4 rates	-	20%
Vomiting · NETTER-1; grade 3-4 rates	-	7%
Nausea · NETTER-1; grade 3-4 rates	-	5%

Rates from the label or pivotal trial as recorded on the product page; each grade 3+ figure links to its source.

Everolimus all recorded rates →

Avoid grapefruit. Live vaccines are contraindicated.
7.5 mg (mild), 5 mg (moderate), 2.5 mg (severe).

Sunitinib all recorded rates →

Avoid grapefruit.
Known QT prolongation. Avoid other QT-prolonging drugs where possible; check ECG and correct potassium and magnesium before and during treatment.

Cabozantinib all recorded rates →

Tablets: take on an empty stomach (no food 2 hours before or 1 hour after). Avoid grapefruit.
Reduce to 40 mg daily in moderate impairment; avoid in severe.

Questions to ask about this decision

Between Lutetium-177 dotatate, Everolimus, Sunitinib and the other options, which do you recommend for me, and what about my case would make you choose differently?
Why: Guidelines list several reasonable options; the choice turns on details of your tumour, your health and your priorities.
What is each option trying to achieve: cure, long control, or relief of symptoms, and over what time?
Why: The aim shapes how much side effect and disruption is worth accepting.
How closely do I match the people in RADIANT-3 and RADIANT-4 and CABINET (Alliance A021602), and does that change what the results mean for me?
Why: Trial populations are selected; age, fitness, prior treatment and biomarkers all affect how far results carry.
Which side effects of Lutetium-177 dotatate are most likely for me, which are reversible, and which would make us stop?
Why: The recorded rates below come from labels and trials; your own risk depends on dose, other medicines and your health.
What happens if I delay, or decline this step for now? Is the decision reversible?
Why: Some decisions can wait for a second opinion or a trial slot; others cannot. Knowing which is part of the choice.
Does your recommendation follow the current guideline (NCCN Guidelines: Neuroendocrine and Adrenal Tumors), and if it departs from it, why?
Why: Departures from guidelines are sometimes right for an individual; they should be explained.
For my situation (progression on a somatostatin analogue), which of the standard options do you recommend and why?
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?
Why: 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?
Why: Trial populations differ from individual patients; ask how closely you match.

Add these to your appointment list, or take the full question set for this cancer.

Other settings

Liver-dominant disease

3 options

Resection, thermal ablation, chemoembolisation or radioembolisation, alongside systemic therapy.

Guideline

NCCN Guidelines: Neuroendocrine and Adrenal Tumors

The options, in plain words

Thermal ablation (RFA, microwave, cryo)Standard of care

Thermal ablation kills a tumour with heat or cold delivered through a needle, with no incision required.

Outpatient, repeatable
Preserves organ function

Transarterial chemoembolisation (TACE)Standard of care

A catheter threaded into the artery feeding a liver tumour delivers chemotherapy and then blocks the vessel, starving the tumour from inside.

Liver-directed with limited systemic toxicity
Decades of evidence and universal availability
Bridges patients to transplant

Radioembolisation (TARE / SIRT, yttrium-90)Established

Millions of tiny radioactive glass or resin beads are injected into the liver artery, lodging in the tumour and irradiating it from within.

Outpatient, single session
Effective in portal vein thrombosis where TACE is contraindicated
Radiation segmentectomy can be curative for small tumours

Also referenced:Hepatectomy (liver resection)Liver-directed therapy (TACE, TARE, HAI, ablation)

The evidence behind it

No trial record is attached to this row yet. The trials tab lists what is recruiting and the landmark trials for this cancer.

The main trade-offs on record

Thermal ablation (RFA, microwave, cryo)

Size limit ~3 cm
Heat-sink near vessels

Transarterial chemoembolisation (TACE)

Post-embolisation syndrome; hepatic decompensation in poor liver function
Rarely curative; repeat sessions
OS benefit of combinations with systemic therapy not yet shown

Radioembolisation (TARE / SIRT, yttrium-90)

Radioembolisation-induced liver disease
Failed to beat sorafenib on OS in advanced disease
Lung shunting excludes some patients

Questions to ask about this decision

Between Thermal ablation (RFA, microwave, cryo), Transarterial chemoembolisation (TACE) and Radioembolisation (TARE / SIRT, yttrium-90), which do you recommend for me, and what about my case would make you choose differently?
Why: Guidelines list several reasonable options; the choice turns on details of your tumour, your health and your priorities.
What is each option trying to achieve: cure, long control, or relief of symptoms, and over what time?
Why: The aim shapes how much side effect and disruption is worth accepting.
What happens if I delay, or decline this step for now? Is the decision reversible?
Why: Some decisions can wait for a second opinion or a trial slot; others cannot. Knowing which is part of the choice.
Does your recommendation follow the current guideline (NCCN Guidelines: Neuroendocrine and Adrenal Tumors), and if it departs from it, why?
Why: Departures from guidelines are sometimes right for an individual; they should be explained.
For my situation (liver-dominant disease), which of the standard options do you recommend and why?
Why: Guideline options include: Resection, thermal ablation, chemoembolisation or radioembolisation, alongside systemic therapy.

Add these to your appointment list, or take the full question set for this cancer.

Other settings

VHL-associated pancreatic NET

One path named

Belzutifan for tumours not requiring immediate surgery (approved 2021).

Guideline

NCCN Guidelines: Neuroendocrine and Adrenal Tumors

The path, in plain words

BelzutifanApprovedSmall-molecule HIF-2α inhibitor

Belzutifan is the first HIF-2α inhibitor, born from Nobel-winning biology, and is now approved after kidney cancer surgery with pembrolizumab.

Also referenced:MEN1 and hereditary neuroendocrine syndromes

The evidence behind it

Belzutifan/MK-6482 for the Treatment of Advanced Pheochromocytoma/Paraganglioma (PPGL), Pancreatic Neuroendocrine Tumor (pNET), Von Hippel-Lindau (VHL Phase 2 NCT04924075355 peopleevidence 33
Tests Belzutifan
A Phase 2 Study to Evaluate the Efficacy and Safety of Belzutifan (MK-6482, Formerly PT2977) Monotherapy in Participants With Advanced Pheochromocytoma/Paraganglioma (PPGL), Pancreatic Neuroendocrine Tumor (pNET), Von Hippel-Lindau (VHL) Disease-Associated Tumors, Advanced Gastrointestinal Stromal Tumor (wt GIST), or Advanced Solid Tumors With HIF-2α Related Genetic Alterations
No headline result recorded yet.

The main trade-offs on record

No side-effect rates or interaction flags are recorded for these options yet. The side-effect lookup and interaction checker cover the products that have them.

Questions to ask about this decision

Is Belzutifan the only reasonable path for me, or is there a trial, a different sequence or a wait-and-see option?
Why: A single standard does not mean a single choice; timing and trials are decisions too.
What is each option trying to achieve: cure, long control, or relief of symptoms, and over what time?
Why: The aim shapes how much side effect and disruption is worth accepting.
How closely do I match the people in Belzutifan/MK-6482 for the Treatment of Advanced Pheochromocytoma/Paraganglioma (PPGL), Pancreatic Neuroendocrine Tumor (pNET), Von Hippel-Lindau (VHL, and does that change what the results mean for me?
Why: Trial populations are selected; age, fitness, prior treatment and biomarkers all affect how far results carry.
What happens if I delay, or decline this step for now? Is the decision reversible?
Why: Some decisions can wait for a second opinion or a trial slot; others cannot. Knowing which is part of the choice.
Does your recommendation follow the current guideline (NCCN Guidelines: Neuroendocrine and Adrenal Tumors), and if it departs from it, why?
Why: Departures from guidelines are sometimes right for an individual; they should be explained.
For my situation (vhl-associated pancreatic net), which of the standard options do you recommend and why?
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?
Why: 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?
Why: Trial populations differ from individual patients; ask how closely you match.

Add these to your appointment list, or take the full question set for this cancer.

How to read this page. Options and results come from OnCo records with their sources; the settings are the standard-of-care rows on the cancer page, and the lines of therapy are on the sequencing grid. Where a setting names one path, the choice is usually about timing, trials and where to be treated: see expert centres. OnCo is orientation, not medical advice.