A blog by Ronny Allan

Treatment for Neuroendocrine Cancer – a summary for patients

Treatment for Neuroendocrine Cancer – a summary for patients

 

1. Scope

This summary provides an overview of the types of therapy known for treating Neuroendocrine Cancer. They will have been approved at least by one national or regional approval agency, may not be available or approved in your own country; and may appear in clinical guidelines for the treatment of Neuroendocrine Cancer.

Clinical trials will not be covered, although it’s noted that some of the approved treatments listed may be in follow on trials either to prove new coverage or used in combination with another drug.  For a list of clinical trials covered by the author, click here.

This summary will not include complementary or alternative treatment but may cover or overlap with experimental treatment.

2. Who recommends the best treatment for my condition? 

Different types of doctors often work together to create a patient’s overall treatment plan that combines different types of treatments. This is called a multidisciplinary team (MDT) (in US the term “tumor board” can be used) and is very important for people with Neuroendocrine Cancer.  These teams may comprise a variety of professionals, such as oncologists, surgeons, gastroenterologists, radiologists, endocrinologists, and many more as permanent members or co-opted for specific cases.

Treatment options and recommendations can depend on several factors, including:

  • The type of Neuroendocrine Neoplasm (NEN) (a term covering well differentiated Neuroendocrine Tumour (NET) and poorly differentiated Neuroendocrine Carcinoma (NEC))
  • Stage and grade
  • The patient’s preferences and overall health
  • The guidelines in place e.g. ENETS, NANETS, NCCN, UKINETS, CommNETS.

3. Treatment vs ‘Watch & Wait’


Watchful waiting or watch-and-wait, sometimes called ‘Active Surveillance, may be recommended for reasons that should always be explained.  Ask why this approach is being taken and what the main risks are – you deserve to know. With this approach, the tumour is closely monitored with regular tests, including but not limited to:

4. Surgery



Surgery is a common treatment for most NETs but less so with NECs. Surgeons, where possible, will try to remove all tumours and most localised NETs are successfully treated with surgery alone, often with curative intent. Additionally, the surgeon will usually remove some tissue surrounding the tumour to achieve a margin between cancerous cells and normal tissue.  Check your post-operative biopsy report to see the details.

In cases of more extensive spread in NETs, a procedure called debulking surgery is sometimes recommended. Debulking surgery removes as much of the tumour bulk as possible (to an extent recommended in local guidelines) and may also provide some relief from symptoms caused by the tumour location and size or by hormonal secretions. Debulking surgery should not be considered curative.  Surgery is not normally considered in NEC (poorly differentiated Neuroendocrine Carcinomas).

Often surgery is deemed not to be possible citing an “inoperable” tumour. Consequently, in these situations, the MDT will recommend another type of treatment. However, some surgeries can be very complex, sometimes risky, and one surgeon’s inoperable could be another’s operable. Many patients will seek a 2nd surgical opinion, always compare risks.

Surgery can be a very different experience from patient to patient. There are different types of NEN, different stages, different ages.  You can read some of my surgery blog posts which contain links and videos with expert comment.

Surgery – to cut or not to cut.  General surgery.

Small Intestine NETs – to cut or not to cut.  The small intestine is not routine bowel surgery.

Small Intestine, Large Surgery.  Patient experience plus expert reviews.

Pancreatic Neuroendocrine Tumours – to cut or not to cut – about surgery for Pancreatic NETs

For GammaKnife/CyberKnife/Stereotactic Radiosurgery (SRS) and SBRT, see Radiation therapy below. For NanoKnife see Ablation Techniques below

5. Somatostatin analogues


Somatostatin is a hormone that controls the release of several other hormones, such as insulin and glucagon. Somatostatin analogues are drugs similar to the hormone  somatostatin and used to control the symptoms created by the hormone-like substances released by a NET. They may also slow the growth of a NET, although they do not generally shrink the tumours.

There are 2 somatostatin analogues used to treat NETs, octreotide (Sandostatin) and lanreotide (Somatuline). Octreotide is available in 2 forms: short-acting is given under the skin (subcutaneously) and long-acting is given as an intramuscular (IM) injection. Lanreotide is given as a long-acting deep subcutaneous injection. The most common side effects are high blood sugars, the development of gallstones, and mild digestive system upset, such as bloating and nausea. There’s actually a third one approved called Pasireotide (Signifor) but that is aimed at Acromegaly and Cushing’s Syndrome.

These drugs work better on somatostatin receptor positive tumours.

Who can get these drugs?  It’s generally approved for those with locally advanced and metastatic cases of Gastroenteropancreatic NETs (GEP NETs) with or without syndrome. In many cases, this will be a first line therapy even administered before surgery in many cases, particularly those with a syndrome.   

Read some of my blog posts about somatostatin analogues:

Lanreotide vs Octreotide – a side by side comparison.

Lanreotide – it’s calling the shots –  my own experience with Lanreotide since 2010.

6. Peptide receptor radionuclide therapy (PRRT)



PRRT is the generic name for a nuclear therapy which uses a radioactive ‘peptide’ to attach to specific ‘receptors’ on a tumour’s cell.  With NETs, the somatostatin receptors which enable this treatment, exist in around 80% of patients.  It’s a treatment not normally offered to poorly differentiated cases which are known not to express these receptors, although trials are ongoing looking for more efficient targets (e.g. DLL-3).

The main treatment used is called Lutathera (the brand name) or Lutetium 177 (Lu177) which indicates the radioactive isotope used to attack tumour cells when combined with a somatostatin analogue variant which leads the payload to the tumour cells due to its known binding effect.  Other brands are coming onstream shortly and/or are in the clinical trials pipeline.

Who can get these drugs?  The treatment is currently only approved for somatostatin receptor positive Gastroenteropancreatic NETs (GEP NETs).  Different healthcare systems may have different patient selection criteria.

Read more here.

7. Targeted therapy (not SSTR targeted)

Targeted therapy is a treatment that targets the tumour’s specific genes, proteins, or the tissue environment that contributes to cancer growth and survival. This type of treatment blocks the growth and spread of tumour cells while limiting damage to healthy cells.

Not all tumours have the same targets.  The main ones in use to treat NETs involve mammalian Target of Rapamycin (mTOR) inhibitors, Tyrosine Kinase Inhibitors (TKI) (including multiple TKIs), Vascular Endothelial Growth Factor (VEGF).  There are several new targeted therapies in the clinical trial pipeline, so it’s worth taking a look at the link above to see more on this type of therapy. These tend to be 2nd or 3rd line treatments, but not always.  

The main targeted therapies currently in use are:

Cabozantinib (approved in many areas 2025/26).  Now approved for advanced pancreatic NET and extrapancreatic NET for age 12 and over.  Read more by clicking here.

Everolimus (Afinitor) is a targeted therapy, an mTOR inhibiter approved for the treatment of advanced non-functional NETs of the GI tract, lung, and pancreas. This drug can help slow down the growth of these tumours in some patients.  Read more by clicking here.

Sunitinib (Sutent) targets a protein called Vascular Endothelial Growth Factor (VEGF), is approved for the treatment of advanced pancreatic NETs. Read more by clicking here.

Who can get these drugs?  It’s generally approved for those with advanced and non-functional cases of Gastroenteropancreatic NETs (GEP NETs) but in the case of Sunitinib, only for pancreatic NETs.

These drugs are not dependent on somatostatin receptors. 

8. Telotristat Ethyl (XERMELO)

Telotristat Ethyl is a significant introduction to the treatment of Carcinoid Syndrome diarrhea. It’s the first addition to the standard of care in more than 16 years and the first-time an oral syndrome treatment has been developed.  The drug was previously known as Telotristat Etiprate but was changed to Ethyl in Oct 2016. ‘Etiprate’ was previously a truncation of ‘ethyl hippurate’.  The brand name is XERMELO®

This drug is approved for the treatment of carcinoid syndrome diarrhea in patients inadequately controlled by somatostatin analogue therapy, i.e. it doesn’t replace somatostatin analogues – it is an additional treatment alongside and only when the diarrhea is caused by hormone oversecretion, i.e. it may not work to prevent post-operative diarrhea or other causal reasons.

In the simplest of terms, the drug is an inhibitor of the enzyme tryptophan hydroxylase (TPH).  TPH is the rate-limiting enzyme in serotonin synthesis which converts tryptophan (an essential amino acid which comes from diet) to 5-hydroxytryptophan, which is subsequently converted to serotonin, one of the main causes of carcinoid syndrome effects including carcinoid heart disease.  The trial data indicates that Telotristat ethyl significantly reduced the frequency of bowel movements.

Read more here

9. Liver‑directed treatment (Updated 2026)

When NETs spread to the liver, several local treatments may be used at different points in the pathway. These procedures are usually performed by an interventional radiologist and may require an overnight stay. They aim to control tumour growth, reduce hormone‑related symptoms, and preserve liver function.  More detailed info than the summary below click here

Why liver‑directed therapy is used in NETs

NET liver metastases are often highly vascular, meaning they rely heavily on blood supply from the hepatic artery. This makes them suitable for treatments that block blood flow or deliver therapy directly into the tumour.

Trans‑arterial therapies (embolisation)

A catheter is guided from the groin or arm into the hepatic artery to target the tumour. The three main types differ only in what is delivered through the catheter.

1. Bland Embolisation (TAE / HAE)

Uses particles to block the tumour’s blood supply.

  • Goal: starve the tumour of oxygen and nutrients.

  • Notes: Often used when chemotherapy is not needed or not helpful.

2. Chemoembolisation (TACE)

Same technique as TAE, but adds chemotherapy (drug varies by country).

  • Notes: Evidence does not clearly show major benefit over bland embolisation for NETs, and side‑effects may be slightly higher.

3. Radioembolisation (TARE / SIRT)

Delivers tiny radioactive beads (usually Yttrium‑90) directly into the tumour’s blood supply.

  • How it works:

    • Mild embolic effect

    • Main effect is targeted internal radiation to the tumour

  • Advantages:

    • Often fewer side‑effects (less post‑embolisation syndrome)

    • Lower risk of liver abscess in patients with previous Whipple/biliary surgery

    • Usually completed in one session

  • Important: Not the same as Y‑90 PRRT, which is a whole‑body systemic therapy.

 

Ablation (covered in its own section)

Some centres may use radiofrequency ablation (RFA) or microwave ablation (MWA) for small, accessible liver lesions. These destroy tumours using heat rather than blocking blood flow. → See the dedicated Ablation section for full details.

 

Special Feature – Histotripsy (emerging technology)

Histotripsy uses focused ultrasound pulses to mechanically break down tumour tissue without heat or radiation.

  • Non‑invasive and image‑guided

  • Currently approved only for liver tumours

  • As evidence grows, it will sit alongside other ablation techniques → See Ablation section for more detail.

 

When liver‑directed therapy may be considered

These treatments are usually considered when:

  • disease is liver‑dominant,

  • symptoms are caused by liver tumours, or

  • the cancer is progressing despite systemic therapy.

Choice of technique depends on tumour biology, liver function, previous surgery, and local expertise.

10. Ablation (Updated 2026)

Ablation destroys tumour tissue directly using heat, cold, electrical pulses, or focused ultrasound. It is usually performed by interventional radiologists and is most commonly used for liver, lung, kidney, and bone lesions. Techniques are broadly divided into thermal and non‑thermal approaches.

🔥 Thermal ablation

Thermal techniques destroy tumour tissue by heating or freezing it.

Radiofrequency ablation (RFA)

Uses electrical current to generate heat and destroy tumour cells. Most effective for small, well‑defined lesions.

Microwave ablation (MWA)

Uses microwaves to produce rapid, predictable heating. Often faster and less affected by tissue impedance than RFA.

Cryoablation

Freezes the tumour using extremely cold probes. Useful when heat could damage nearby structures or when precise control of the ablation zone is needed.

Thermal HIFU (High‑Intensity Focused Ultrasound)

Uses externally applied, focused ultrasound to heat and destroy tissue. Guided by ultrasound or MRI. Completely non‑invasive, but its use is limited by bone, bowel gas, and tumour depth.

🔥Where thermal ablation works best: Commonly used for liver metastases and selected lung or bone lesions.

 

⚡ Non‑thermal ablation

Non‑thermal techniques destroy tissue without heat, which helps preserve nearby structures and avoids the “heat‑sink” effect caused by blood flow.

Irreversible electroporation (IRE)

Delivers short electrical pulses that permanently disrupt cell membranes. Particularly useful near major blood vessels, bile ducts, or other sensitive structures where heat would be unsafe.

Histotripsy

A newer, non‑invasive technique using focused ultrasound pulses to create microbubbles that mechanically break apart tumour tissue. Guided by real‑time ultrasound. Currently used mainly for liver tumours, with research expanding into kidney, pancreas, and other organs.

 

🎯 Where ablation fits in NET treatment

Ablation can be a valuable local treatment option for people with neuroendocrine tumours (NETs), especially when:

  • Liver metastases — most common use; works best for small, accessible lesions.

  • Lung lesions — selected cases depending on size and location.

  • Bone lesions — cryoablation can help with pain control and local tumour control.

  • Kidney or adrenal lesions — depending on size, accessibility, and proximity to critical structures.

Ablation is not a replacement for systemic therapy, but it can be an effective option when:

  • Surgery is not possible.

  • Only a small number of lesions need control.

  • Local progression threatens symptoms or organ function.

  • A “debulking” approach is considered as part of a multidisciplinary plan.

Read my Ablation Blog

Click the picture to read more

11. Chemotherapy (updated 2026)



Chemotherapy plays very different roles depending on the type of NEN.

🟦 Well‑differentiated NETs (G1–G3)

Chemo is not the main treatment for most NETs, but it can be very useful in specific situations — especially for pancreatic NETs and higher‑grade NETs that are growing more quickly.  Commonly used options include:

  • CAPTEM (capecitabine + temozolomide)

  • FOLFOX (5‑FU + oxaliplatin)

  • CAPOX/CAPEOX/XELOX (capecitabine + oxaliplatin)

  • Streptozotocin‑based regimens

Chemo may be used:

  • When disease is progressing

  • When tumour shrinkage is needed

  • As neoadjuvant therapy in selected pancreatic NETs

  • Occasionally after surgery in high‑risk cases

Most low‑grade midgut NETs do not respond well to chemotherapy.

🟥 Poorly differentiated NECs

This is a completely different disease, and chemotherapy is essential first‑line treatment.

The standard regimen is:

  • Platinum + etoposide (cisplatin or carboplatin with etoposide)

Other regimens such as FOLFIRI or FOLFOX may be used later if the cancer returns or stops responding.

 

🧠 Key point

Chemotherapy is not one-size-fits-all in NENs. It is selective and strategic in NETs, but often mandatory and urgent in NECs.

Read more about chemotherapy here.


12. Immunotherapy

Image result for immunotherapy

Immunotherapy is designed to boost the body’s natural defences to fight the tumour. It uses materials made either by the body or in a laboratory to improve, target, or restore immune system function. Sometimes it is called biologic therapy.

There’s actually been an immunotherapy drug used in Neuroendocrine Neoplasms for some time and some of the long-term patients may have heard of it or have had it administered.  Known as Interferon alpha (IFNα) it helps the body’s immune system work better and can lessen diarrhea and flushing. It may also shrink tumours.  I still see this listed in some texts but less so recently, probably due to low efficacy and high toxicity.

When people talk about Immunotherapy today, they really mean the new drugs.  While there are many clinical trials (some of which have already been declared not suitable), only two Neuroendocrine Neoplasms have at least one modern Immunotherapy drug approved:

Read more about Immunotherapy for Neuroendocrine Cancer here.  Given the amount of immunotherapy drugs in clinical trials, might be useful to take a look at all the posts I’ve written about clinical trials in the link above.


13. External Beam Radiation Therapy (EBRT) (Updated 2026)

Image result for radiotherapy

EBRT uses high‑energy beams to damage cancer cells. In neuroendocrine tumours (NETs), it is used selectively depending on tumour type, location, grade, and the overall treatment plan. Modern radiotherapy is far more precise than older techniques, and new technologies now allow safe treatment of tumours that were previously difficult to target. Note: This section covers EBRT, which uses machines outside the body to target tumours. Peptide Receptor Radionuclide Therapy (PRRT) is a different type of radiotherapy delivered internally via a cannula and was explained separately above. 

External Beam Radiotherapy can be understood in three layers: the technique, the platform, and the imaging guidance.  The reason I include these distinctions is that the naming conventions used get mixed up particularly inside patient support groups where a patient/caregiver uses the technique or the platform or the technique in different contexts, i.e. ‘synonym soup’.

1. Radiotherapy techniques (what patients usually hear about)

These describe how the radiation dose is delivered.

  • IMRT / VMAT — shape the radiation beam to match the tumour while sparing nearby organs.

  • SBRT (Stereotactic Body Radiotherapy) — very precise, high‑dose treatment delivered in a small number of sessions. Effective for bone, lung, liver, and some adrenal NETs.

  • Adaptive radiotherapy — the plan is adjusted daily based on that day’s anatomy. Useful for tumours that move or sit close to sensitive organs.

  • IMPT (Intensity‑Modulated Proton Therapy) — the proton‑based equivalent of IMRT, used in selected cases.

These techniques can be delivered on different machines depending on the clinical situation.

2. Radiotherapy platforms (the machine that delivers the beam)

These machines generate and shape the radiation beam. They differ in precision, imaging capability, and suitability for soft‑tissue tumours.

  • Conventional LINAC (linear accelerator) The most common radiotherapy machine worldwide. Delivers high‑energy X‑rays (photons). Suitable for many NET sites, especially bone, lung, and some liver lesions.

  • CyberKnife (robotic LINAC) A specialised LINAC mounted on a robotic arm. Delivers photon radiotherapy with sub‑millimetre precision and real‑time X‑ray tracking. Often used for lung, spine, prostate, and selected liver lesions. It is a platform, not a technique — it delivers SBRT rather than replacing it.

  • MRI‑LINAC (MRI‑guided radiotherapy) Combines a LINAC with real‑time MRI. Allows continuous visualisation of soft tissues and daily adaptive planning. Particularly useful for tumours that move or sit close to sensitive organs — such as pancreatic NETs.

  • Proton therapy systems Use charged particles (protons) instead of X‑rays. These machines are cyclotrons or synchrotrons, not LINACs. Protons deposit most of their energy at a specific depth (the Bragg peak), reducing dose to surrounding tissues. Best suited to fixed, predictable anatomy (brain, skull base, paediatrics). Less commonly used for mobile abdominal organs like the pancreas.

3. Imaging guidance (how the tumour is seen and tracked)

Imaging determines how accurately the tumour can be targeted.

  • Cone‑beam CT (CBCT) — standard on most LINACs; good for bone and lung but limited for soft‑tissue organs such as the pancreas or bowel.

  • X‑ray tracking — used by CyberKnife to follow tumour motion, often with implanted fiducials.

  • MRI guidance — used on MRI‑LINACs; provides clear soft‑tissue visibility and real‑time tracking. Allows the beam to pause automatically if the tumour moves.

Where radiotherapy fits in NET treatment

You won’t find much of this in guidelines but there are studies and suggested used in papers.  For example SBRT is not listed as a standard of care in any major NEN guideline, including NCCN, ENETS, NANETS, or ESMO. This is because radiotherapy plays a selective, rather than central, role in NET management. That said, SBRT is mentioned in the NCCN guidelines in several site‑specific contexts, and there is a growing body of NET‑specific evidence supporting its use in carefully chosen situations.  Click here to read more about SBRT in Neuroendocrine Cancer.

14. Summary of this whole article

I wanted to provide a summary of the main treatments for Neuroendocrine Cancer without going into too much detail but still delivering something of use to the average patent. Not everyone will have access or need access to all of these treatments.  I’m sure this is not an exhaustive list and I’m happy to listen to feedback on areas of both the quality and quantity of the information.

If you are in need of further information about your own treatment options, you should of course speak directly to your specialist. If you need to hear the experience of others who have gone through these treatments, you should post in my group.  For those wishing to join, there’s a green button below with a link.
Beware of snake oil stuff online – click picture to read more

 

Disclaimer

I am not a doctor or any form of medical professional, practitioner or counsellor. None of the information on my website, or linked to my website(s), or conveyed by me on any social media or presentation, should be interpreted as medical advice given or advised by me.  Neither should any post or comment made by a follower or member of my private group be assumed to be medical advice, even if that person is a healthcare professional. Please also note that mention of a clinical service, trial/study or therapy does not constitute an endorsement of that service, trial/study or therapy by Ronny Allan, the information is provided for education and awareness purposes and/or related to Ronny Allan’s own patient experience. This element of the disclaimer includes any complementary medicine, non-prescription over the counter drugs and supplements such as vitamins and minerals.

General Clinical Trials Disclaimer

Choosing to participate in a study is an important personal decision. Talk with your doctor and family members or friends about deciding to join a study. To learn more about this study, you or your doctor may contact the study research staff using the contacts provided in the clinical trials document. It’s very important to check the trial inclusion and exclusion criteria before making any contact.  If you need questions, the articles here is very useful Questions to Ask About Clinical Trials | Cancer.Net

The inclusion of any trial within this blog should not be taken as a recommendation by Ronny Allan.


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Thanks for reading

Ronny



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By Ronny Allan

Ronny Allan is a 3 x award-winning accredited patient leader advocating internationally for Neuroendocrine Cancer and all other cancer patients generally. Check out his Social Media accounts including Facebook, BlueSky, WhatsApp, Instagram and and X.

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