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Living with cancer·Standard of care

Grade 3 well-differentiated neuroendocrine tumour: the decisions you may face

Cancer page →Second-opinion finder →

5 treatment settings, 4 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.

2 options

Morphology, Ki-67 and p53 or Rb immunohistochemistry to separate grade 3 tumour from carcinoma; somatostatin receptor and FDG PET together.

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
PET (positron emission tomography)Standard of care

A scan that shows where a radioactive tracer accumulates, so it images what tumours are doing rather than what they look like.

  • Whole-body biology in one scan
  • Quantitative
  • Any target with a ligand can in principle be imaged
Also referenced:Neuroendocrine tumour grade (Ki-67) and WHO classificationTumour differentiation (well / moderately / poorly differentiated)TP53
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
PET (positron emission tomography)
  • Resolution ~4 mm
  • Tracer supply and cost
  • Inflammation confounds FDG
Questions to ask about this decision
  1. Between Somatostatin receptor PET (68Ga/64Cu-DOTATATE) and PET (positron emission tomography), 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.
  2. 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.
  3. 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.
  4. 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.
  5. For my situation (diagnosis), which of the standard options do you recommend and why?
    Why: Guideline options include: Morphology, Ki-67 and p53 or Rb immunohistochemistry to separate grade 3 tumour from carcinoma; somatostatin receptor and FDG PET together.

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

Advanced, first line

Advanced, somatostatin receptor-positive

3 optionsCompare side by side →

Lutetium-177 dotatate first line for Ki-67 up to 55 percent (NETTER-2); somatostatin analogue alongside.

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.

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
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.

The evidence behind it
The main trade-offs on record
Lutetium-177 dotatateall recorded rates →
Side effectAny gradeGrade 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
  1. Between Lutetium-177 dotatate, Peptide receptor radionuclide therapy (PRRT) and Somatostatin analogues (octreotide, lanreotide), 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.
  2. 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.
  3. How closely do I match the people in 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.
  4. 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.
  5. 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.
  6. 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.
  7. For my situation (advanced, somatostatin receptor-positive), which of the standard options do you recommend and why?
    Why: Guideline options include: Lutetium-177 dotatate first line for Ki-67 up to 55 percent (NETTER-2); somatostatin analogue alongside.
  8. Am I a candidate for Lutetium-177 dotatate, Somatostatin analogues (octreotide, lanreotide), and what side effects should I expect?
    Why: Knowing the expected toxicities helps you plan work, family, and supportive care.
  9. How do the results of 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.

Advanced, first line

Advanced, shrinkage needed or receptor-negative

4 optionsCompare side by side →

Capecitabine with temozolomide; everolimus or sunitinib for pancreatic tumours; platinum-etoposide for carcinoma-like tempo or Ki-67 near 55 percent.

Guideline
NCCN Guidelines: Neuroendocrine and Adrenal Tumors
The options, in plain words
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.

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.

Platinum + etoposide (EP / CE)Standard of careCytotoxic regimen

Platinum plus etoposide has been the chemotherapy backbone of small-cell lung cancer for over 40 years, and is now given with immunotherapy.

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
Everolimusall recorded rates →
  • Avoid grapefruit. Live vaccines are contraindicated.
  • 7.5 mg (mild), 5 mg (moderate), 2.5 mg (severe).
Sunitiniball 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.
Questions to ask about this decision
  1. Between Capecitabine + temozolomide (CAPTEM), 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.
  2. 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.
  3. 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.
  4. 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.
  5. For my situation (advanced, shrinkage needed or receptor-negative), which of the standard options do you recommend and why?
    Why: Guideline options include: Capecitabine with temozolomide; everolimus or sunitinib for pancreatic tumours; platinum-etoposide for carcinoma-like tempo or Ki-67 near 55 percent.
  6. Am I a candidate for Capecitabine + temozolomide (CAPTEM), 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.

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

One path named

COMPOSE: 177Lu-edotreotide against CAPTEM, everolimus or FOLFOX in aggressive grade 2 and grade 3 gastroenteropancreatic tumours.

Guideline
NCCN Guidelines: Neuroendocrine and Adrenal Tumors
The path, in plain words
177Lu-edotreotidePhase 3Radioligand therapy (beta)

A second lutetium radioligand for neuroendocrine tumours that beat the standard pill everolimus in a head-to-head trial and is awaiting an FDA decision.

The evidence behind it
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
  1. Is 177Lu-edotreotide 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.
  2. 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.
  3. How closely do I match the people in Lutetium 177Lu-Edotreotide Versus Best Standard of Care in Well-differentiated Aggressive Grade-2 and Grade-3 GastroEnteroPancreatic NeuroEndocrine Tumors (GEP-NETs) - COMPOSE, 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.
  4. 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.
  5. 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.
  6. For my situation (trials), which of the standard options do you recommend and why?
    Why: Guideline options include: COMPOSE: 177Lu-edotreotide against CAPTEM, everolimus or FOLFOX in aggressive grade 2 and grade 3 gastroenteropancreatic tumours.
  7. Am I a candidate for 177Lu-edotreotide, and what side effects should I expect?
    Why: Knowing the expected toxicities helps you plan work, family, and supportive care.
  8. How do the results of Lutetium 177Lu-Edotreotide Versus Best Standard of Care in Well-differentiated Aggressive Grade-2 and Grade-3 GastroEnteroPancreatic NeuroEndocrine Tumors (GEP-NETs) - COMPOSE 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

Limited disease

2 options

Resection and liver-directed therapy as for other well-differentiated tumours.

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
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
Questions to ask about this decision
  1. Between Thermal ablation (RFA, microwave, cryo) and Transarterial chemoembolisation (TACE), 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.
  2. 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.
  3. 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.
  4. 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.
  5. For my situation (limited disease), which of the standard options do you recommend and why?
    Why: Guideline options include: Resection and liver-directed therapy as for other well-differentiated tumours.

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.