A blog by Ronny Allan

Treatment for Neuroendocrine Cancer – a summary for patients

Treatment for Neuroendocrine Cancer – a summary for patients

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.

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/UKINETS/CommNETS)

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:

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), see Radiation therapy below. For NanoKnife see Ablation Techniques below

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.

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

Peptide receptor radionuclide therapy (PRRT)


PRRT is the generic name for a nuclear therapy which uses a radioactive ‘peptide’ to attach to 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.

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.

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.

Targeted therapy

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 and Tyrosine Kinase Inhibitors (TKI).  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.

The main targeted therapies currently in use are:

Cabozantinib (approved March 2025).  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 here.

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

Liver-directed treatment


If cancer has spread to the liver, the treatments below may be used at some point.  These procedures are usually performed by an experienced interventional radiologist and may require an overnight hospital stay.

  • Liver Embolization.  NETs are known to be highly vascular, that is they live on blood supply. Tumour embolization is a procedure to shrink a liver tumour by cutting off its blood supply. The doctors put a thin, flexible tube, called a catheter, into an artery near your groin or in your arm. He or she guides the catheter into the liver artery (the hepatic artery) that supplies blood to the tumour. So, the theory is if you block the blood supply to a tumour, the tumour will reduce or die. It’s a procedure carried out by an interventional radiologist – it’s very much image guided. Other countries may use other appropriately trained personnel and they may have different names.  There are essentially 3 types of liver embolization: Bland – Often known as Hepatic Arterial Embolization (HAE) or TransArterial Embolization (TAE). The technique is as described above.
    Chemo – Transarterial Chemo Embolization (TACE).     As per the bland type but they add some chemo (normally Fluorouracil (5-FU) but could be different depending on where you are). There is currently some debate about the actual advantages of that over bland.

    Radioembolization – Transarterial Radio Embilization (TARE).  Also often called selective internal radiotherapy (SIRT), uses tiny radioactive beads (microspheres). It damages the blood vessels to the tumour so that it can’t get the nutrients it needs, and the radiation given off by the SIRT beads damages the cancer cells and hopefully stops them growing. The radiation used is mostly always Yttrium 90 or Y90. (Should not be confused with the Y90 version of Peptide Receptor Radio Therapy (PRRT) which is a different type of treatment and for the whole body).

Read more here.

Special Feature – Histotripsy – read more here

Ablation Techniques


Radiofrequency ablation (RFA) and microwave ablation (MWA) use heat to destroy cancer cells or tumours (more than just the liver). The heat is supplied by electrical currents passed through a special needle placed directly into the liver. This may also be known as a percutaneous ablation. RFA/MWA is undertaken by doctors with special experience, and it may not be available at all treatment centres.

Cryoablation for cancer is a treatment to kill cancer cells with extreme cold. During cryoablation, a thin, wand-like needle (cryoprobe) is inserted through your skin and directly into the cancerous tumour. A gas is pumped into the cryoprobe in order to freeze the tissue. Then the tissue is allowed to thaw. The freezing and thawing process is repeated several times during the same treatment session.

Cryoablation for cancer may be used when surgery isn’t an option. Cryoablation is sometimes used as a primary treatment for:

  • Bone cancer
  • Cervical cancer
  • Eye cancer
  • Kidney cancer
  • Liver cancer
  • Lung cancer
  • Prostate cancer

Cryoablation is also used to relieve the pain and other symptoms caused by cancer that spreads to the bone (bone metastasis) or other organs.

Cryoablation for cancer may also be called percutaneous cryoablation, cryosurgery or cryotherapy.

Irreversible electroporation also known as IRE, is a non-thermal ablative technique used to destroy cancer cells. It is a relatively new technique that has been used in medicine for over a decade, and its use is not widespread, as research is still being done into its efficacy. It is potentially a way to stop the growth or even reduce the size of cancer and prevent it from spreading (metastasis). The most well known version is branded under the name of Nanoknife® Today, the cancer most commonly treated with IRE is stage III pancreatic cancer. It can also be used in some cases for liver cancers (colorectal liver metastases, hepatocellular carcinoma (HCC), and neuroendocrine tumours) that cannot be treated with surgical resection (liver resection). Some urological cancers including prostate cancer and renal cell cancer may also benefit from treatment with IRE.  IRE is based on pulses of electrical energy delivered between two electrodes. These electric pulses affect the membranes (outer surface) of the cancerous cells, changing the existing cellular membrane potential. This results in nanoscale (tiny) defects in the lipid bilayer of the membrane, which disrupts the stability of the cells and leads to a phenomenon known as apoptosis – cell death controlled by the body itself. The extent of cell damage and death depends on the amplitude, duration, frequency and number of pulses applied. Because IRE causes cell death through apoptosis, structures formed by proteins, such as vascular elastin and collagenous structures, are not affected, and therefore surrounding blood vessels are preserved. This allows for the ablation of malignancies (cancers) that are surrounded by these structures, which is typically the case for locally advanced pancreatic cancer. Unlike thermal-induced methods, such as microwave and radiofrequency ablation, which result in fibrosis and scarring, the apoptotic cells are phagocytosed (destroyed) by cells of the immune system and replaced by innate cellular degeneration.  This treatment is not widely available.

Other less common methods

There are several other ablation techniques used worldwide: including Interstitial laser ablation and high-intensity focused ultrasound (HIFU).

Histotripsy – read more here

Read my Ablation Blog

Includes the world’s first to use a recently FDA-approved ablation technology that can destroy large liver tumours.

Click the picture to read more

Chemotherapy


Chemotherapy (chemo) is the use of drugs to destroy cancer cells, usually by keeping the cancer cells from growing, dividing, and making more cells. When people talk about “Chemo”, particularly patients and caregivers, they will anticipate this being the cytotoxic type of therapy commonly associated with toxic side effects including hair loss as the most well-known one.  To a certain extent, this is accurate but worth pointing out that some chemo is more toxic than others (but still necessary depending on cancer type), some chemo is oral (tablet form) and not everyone suffers hair loss, and side effects usually go away after treatment is finished.  Chemotherapy is normally administered via intravenous route (IV) but there are also oral (tablet) forms and may also be injected through a catheter (thin tube) directly into the abdominal cavity. This is called intraperitoneal (IP) chemotherapy.  The latter is only for specific areas of anatomy.

Who gets Chemo in Neuroendocrine Neoplasms (NEN)?

There’s a longstanding myth in patient groups that “it doesn’t work with NETs”.  This is only partly true.  It is known not to be sufficiently effective with low grades (but still may be given in certain scenarios), it does not appear to like its slow cytokinetic growth.  However, on the other end of the spectrum, it’s normally a first-line treatment for poorly differentiated Neuroendocrine Carcinomas.

It may also be a strong option for well-differentiated Grade 3 NETs and in certain scenarios for Grade 2 NETs.

Chemo types:

Platinum-based drugs (informally called platins) are chemotherapeutic agents used to treat many types of cancer including Neuroendocrine where it is tends to be exclusively for poorly differentiated Neuroendocrine Carcinoma. These drugs are used to treat almost half of people receiving chemotherapy for cancer. Commonly used chemo includes Cisplatin, Oxaliplatin, and Carboplatin.

Other Cytotoxic chemo include:

  • Fluorouracil (5-FU),
  • Streptozotocin (Zanosar)
  • Irinotecan (Campto)
  • Paclitaxel (Taxol)

and the oral (tablet) versions:

  • Capecitabine (Xeloda),
  • Temozolomide (Temodal),
  • Etoposide (Etopophos, Vepesid)

Known combos used in NENs.

Cisplatin and Etoposide appear to be the most commonly used combo for high grade.

Capecitabine (Xeloda) and Temozolomide (Temodal) (CAPTEM) are used in cases of Grade 2 and Grade 3 NET but also listed as a second- or third-line treatment for NEC.  Read more about CAPTEM by clicking here.

FOLFOX. Also known as Oxaliplatin de Gramont or OxMdG, which means modified Oxaliplatin de Gramont. It is made up of folinic acid (also called leucovorin, FA or calcium folinate), fluorouracil (5FU) and oxaliplatin.

FOLFIRI.  Also known as irinotecan de Gramont or irinotecan modified de Gramont. It includes folinic acid (also called leucovorin, calcium folinate or FA), fluorouracil (5FU) and irinotecan.

Oxaliplatin and capecitabine (XELOX) Oxaliplatin and capecitabine is a chemotherapy combination known as XELOX. This combination might also be called CAPOX, CAPE-OX or OxCap.

FCarboSt. A combo of Fluorouracil, Streptozocin and Carboplatin.  Use may be limited to Royal Free Hospital London.  I would love to hear from others.

XELIRI or CAPIRI.  Capecitabine and Irinotecan.

Read more about chemotherapy here.

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.


Radiation therapy (Updated 2026)

Image result for radiotherapy

Radiotherapy 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.

Radiotherapy can be understood in three layers: the technique, the platform, and the imaging guidance.

 

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

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. However, 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.

 

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


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.

Click here to enter Ronny’s Clinical Trials Archive

 


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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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