Also known as:
- Neuroendocrine neoplasms (NENs)
- or historically as carcinoid tumours
A word about naming (terminology).
This is a very diverse group of cancers, originating in a specific type of cell, present in and affecting many different organs and sites of the body – with no typical pattern of behaviour.
Neuroendocrine Cancers have had a variety of names since they were first identified in 1907, however, it has been agreed, by expert consensus – including the World Health Organisation (2017) – that they are called Neuroendocrine Neoplasms (NENs) : after the type of cell that they form in.
Neuroendocrine cells are found throughout the body and may produce certain hormones, or similar substances, to help body systems function healthily.
There are 2 distinct classifications of NENs:
- Neuroendocrine Tumours (NETs): cells have an appearance, seen under the microscope, described as well-differentiated. NETs can have either have different rates of cell division and growth rate – from slow (Grade 1), to moderate (Grade 2) to rapid (Grade 3).
- Neuroendocrine Carcinomas (NECs): cells have an appearance, seen under the microscope, described as poorly-differentiated. NECs can further be described as being either small cell or large cell and have a rapid cell division and growth rate (equivalent or higher than Grade 3 NET).
Please note: that at time of writing old terms may still be in use for certain primary sites, for example, in Lung NENs, the terms Typical and Atypical Carcinoid Tumours may be used to described low to rapid grade Lung NETs. However, Lung NECs will be referred to based on either they have a small or large cell appearance: small cell Lung NECs are abbreviated to SCLC and large cell Lung NECs are abbreviated to LCNECs.
NENs may also be further classified according to:
- The organ it may start in : for example Small Bowel NET or NEC
- The hormone it might over produce : for example an insulinoma is a pancreatic NET that releases too much insulin. NECs rarely over-produce site specific hormones.
In this information we will use the following terms and abbreviations :
- Neuroendocrine Cancers or NENs
- Neuroendocrine Tumours or NETs
- Neuroendocrine Carcinomas or NECS
What neuroendocrine cancer is not.
The most common cancer is adenocarcinoma – a cancer that starts in mucus-producing (glandular) cells. Many parts of the body have these types of cells and adenocarcinoma can develop in any of these organs. Adenocarcinoma is a very large group of cancers – and is usually classified by site of origin e.g. breast, lung, bowel or pancreas – and often uses the common term for malignancy: ‘cancer’.
But it is not the only cancer – there are others, that develop in other types of cell , for example, sarcomas and neuroendocrine cancers (NENs).
Neuroendocrine Cancers (NENs) are not a type of adenocarcinoma – they start in neuroendocrine cells – they can have different signs and symptoms to other cancers, different tests required to diagnose them, different treatments to cure or manage them and different outcomes.
The need for accurate diagnosis – and terminology – is vital, and can help people to access appropriate and accurate disease information and support, and appropriate and accurate expert advice, investigation and treatment.
Both of which can positively influence experience of care and quality of life.
Contents
How rare are neuroendocrine cancers (NENs)?
In the UK and Europe the usual definition of a less common cancer is one that affects less than 12 people out of 100,000 each year – and a rare cancer is one that occurs in less than 6 people out of every 100,000 each year.
In the UK, the 4 most common cancers are Lung, Bowel, Breast and Prostate Adenocarcinomas – and these accounted for over half (53%) of all new cancer cases in the UK in 2016-2018, but less than half (45%) of all cancer deaths in the UK in 2017-2019.
There are around 200 rare cancers and they make up about 24% (nearly a quarter) of all new cancer diagnoses each year.
According to recent figures, Neuroendocrine Cancers, as a group, have an incidence of 8.8:100,000: that is about 5,000 people, each year being newly diagnosed (2018: England).
However, when we look more closely at this group, we can see that it is a collective of distinctly different rare and ultra-rare cancers – different sites, signs, symptoms and patterns of cancer behaviour.
As a group, Neuroendocrine Cancers, have a reported prevalence (number of people currently living with this diagnosis) of 48:100,000: that is, approximately 27,000 people (England, 2018)
NENs are seen in people of all ages, ethnicity, sex, gender, income, geographical location and educational background.
Although they may occur almost anywhere in the body, the most common primary sites are the lungs, pancreas and small bowel.
The incidence of individual neuroendocrine cancers is summarised in the separate information sheets.
What are neuroendocrine cancers (NENs)?
Cancer is a condition where cells within the body become abnormal and start to divide, replicate and grow uncontrollably.
Solid organ cancer cells can invade and destroy surrounding healthy tissue, including nearby organs and structures. Some cancer cells can spread from their original site (primary) to other parts of the body – by direct invasion or through the blood and lymphatic system — leading to secondary sites of disease (called secondaries or metastases).
Note: Blood and lymphatic cancers (non-solid organ cancers) may develop and spread slightly differently as the systems in which they start are fluid.
Neuroendocrine cancers develop in solid organs, including the skin, and occur when neuroendocrine cells become abnormal, start to grow and / or behave in an uncontrolled way – this can affect the healthy working of one or more body systems, such as the digestive system.
What are neuroendocrine cells?
Our bodies are made up of billions of cells including neuroendocrine cells. In order for our bodies to function correctly, messages have to be sent between different organs and tissues, for example, instructions on how to breathe, prepare for ‘fight or flight’, make more or less digestive juices (in response to food) and to control sugar levels in the bloodstream.
These messages can be sent through nerves (by your neurological system), through the release of hormones (by your endocrine system) or through a combination of both (by your neuroendocrine system). As with any message there needs to be a sender and a receiver.
Neuroendocrine cells are found all over the body, in quite high numbers in key organs such as the small bowel, lungs and endocrine glands, such as the pancreas. They are also present in lower, less densely packed numbers, amongst cells of the skin and other body sites and organs.
In health, neuroendocrine cells, help regulate our bodily functions by releasing hormones or small molecules that have a similar effect: they send messages, release hormones and have specific receptors to receive message and hormones.
When neuroendocrine cells become cancerous, this ability to release these substances can become disrupted leading to excess production and release.
In Neuroendocrine Tumours, particularly Grades 1-2, the receptors can become more evident (the medical term is ‘over-expression’) and this can provide a target for nuclear medicine diagnostic scans and certain treatments.
In Neuroendocrine Carcinomas, these receptors may disappear, but the higher activity of cells, can be seen on a different nuclear medicine scan than used in NETs. This scan can provide information to inform diagnosis and treatment choice.
Note: Grade 3 NETs may have either over-expressed receptors or none at all, and depending on rate of growth, (Ki67%) may also benefit from undergoing similar scans to those used in NECs.
What are hormones?
Hormones are necessary for many of the body’s normal activities to occur. They are vital to our growth and development, our appetite and digestion, activity and sleep – and also play a role in our immune response and mental health.
If our hormone levels are abnormally increased or decreased this can result in a number of symptoms or syndromes occurring that can affect both our physical and psychological well-being.
According to the Society for Endocrinology:
A hormone is a chemical that is made by specialist cells*, usually within an endocrine gland, and it is released into the bloodstream to send a message to another part of the body.
In the human body, hormones are used for two types of communication:
The first is for communication between two endocrine glands, where one gland releases a hormone which stimulates another target gland to change the levels of hormones that it is releasing.
The second is between an endocrine gland and a target organ, for example when the pancreas releases insulin which causes muscle and fat cells to take up glucose from the bloodstream.
https://www.yourhormones.info/hormones
* specialist cell refers to both endocrine and neuroendocrine cells.
Neuroendocrine cells can communicate with each other in the same way as explained above.
So, although hormones can circulate throughout the body, coming into contact with many different types of cell, they can only affect cells that have specific hormone receptors.
Cells may have many receptors for a specific hormone or receptors for more than one type of hormone. The number of receptors may also change over time – either increasing or decreasing the cell’s sensitivity to a particular hormone.
What is neuroendocrine cancer?
Neuroendocrine cancers occur when neuroendocrine cells become abnormal and begin to divide or behave in an uncontrolled manner.
Signs and symptoms may depend on the classification : NET or NEC.
They may also depend on:
- the size, shape and location of the cancer: for example, if it is blocking a tube (e.g. airway) or pressing on its original or a nearby organ or body structure (e.g. blood or lymphatic vessel) .
- by the changes caused by the hormones produced by the tumour: for example an insulinoma – which produces abnormally high levels of insulin, causing abnormally low blood sugar levels.
Image: Design by Teresa Ruggle @University of Iowa
Describing different types of neuroendocrine cancer behaviour
By cell appearance and growth rate
As previously mentioned, there are 2 distinct classifications of NENs:
- Neuroendocrine Tumours (NETs): cells have an appearance, seen under the microscope, described as well-differentiated. NETs can have either have different rates of cell division and growth rate – from slow (Grade 1), to moderate (Grade 2) to rapid (Grade 3).
- Neuroendocrine Carcinomas (NECs): cells have an appearance, seen under the microscope, described as poorly-differentiated. NECs can further be described as being either small cell or large cell and have a rapid cell division and growth rate (equivalent to or higher than Grade 3 NET).
This distinction is determination by differentiation: cell appearance. The less well differentiated a cancer cell is, the less like it’s original cell it is and the more likely it is to behave aggressively.
Differentiation refers to how different the cancer cell looks compared to what it should look like as a normal cell. Using this information, it is possible to divide neuroendocrine cancers into the two key classifications of NET or NEC.
Grading can also help predict how slowly or rapidly the cancer may grow and/or spread, based on checking how many active abnormal cells are contained within a cancerous growth.
Mitotic rate: measures how fast cancer cells are dividing and growing. To find the mitotic rate, the number of cells actively dividing in a certain amount of cancer tissue is counted. This may be reported in histopathology reports using the abbreviation MiB-1 or Mitoses per 10 HPF.
However, for most neuroendocrine cancers, histopathologists use Ki67 to ‘stain’ or identify actively dividing cancer cells. You may see the term Ki67 followed by a percentage used in your clinical letters or reports.
Ki67 is a protein that is present during all of the active stages of the cell cycle, a valuable marker of proliferation (cell division and growth), often expressed as a percentage(%). The higher the Ki67%, the more rapidly the cells divide and grow.
Broadly speaking there are 4 grading categories of Neuroendocrine Cancer:
- Grade 1 is a well-differentiated cancer with a Ki67 less than 3%. (PHOTO A)
- Grade 2 is a well-differentiated cancer with a Ki67 between 3 and 20%. (PHOTO B)
- Grade 3 is a well-differentiated cancer with a Ki67 more than 20%. (PHOTO C)
- The 4th category is NEC – a poorly-differentiated cancer with a Ki67 more than 20%, often above 50%. (PHOTO D)
Image Source: Cuthbertson, Shankland & Sririjaskanthan (2023) Clin Med
Essentially the higher the Ki67% the more rapid or aggressive the cancer may behave – and those that are also poorly-differentiated may be the most difficult to control.
The importance of accurate grading cannot be over-emphasised as it is a key determinant of both treatment planning and decision-making, alongside prognosis (where the prognosis is the prediction of the most likely course of the disease as well as the treatment response).
By cell functionality
Healthy neuroendocrine cells release hormones, but when they become cancerous they may release higher levels of hormone than before. This affects whether or not the cancer produces certain symptoms. It may also affects the way it is treated. The effects of too much hormone may need to be treated before or alongside the cancer.
- If the cancer produces uncontrolled, higher than normal, levels of hormone, it is called a functional neuroendocrine tumour.
- If the cancer produces usual or lower levels of hormone it is called a non-functional neuroendocrine tumour.
The majority of NENs are non-functional: and those that are functional are predominantly Neuroendocrine Tumours.
Please note: some NENs may produce hormone-related symptoms, such as Hypercalcaemia or Cushing’s as part of a paraneoplastic syndrome – this is a rare and separate consequence of diagnosis to that discussed above, and if seen, is more common in NECs, than NETs.
Risk factors:
For many Neuroendocrine Cancers there is no obviously identifiable direct cause or risk factor.
However, NECs may have similar risk factors as more common cancers, eg smoking.
For example, this is an identifiable risk factor for Lung NEC (both small and large cell) – however there is less evidence that smoking is a similar factor in Lung NET (particularly the low grade Typical Carcinoid group): with many diagnosed having no smoking history at all.
Genetic factors – some rare, inherited, conditions can increase the risk of Neuroendocrine Cancer development: more commonly NET, though NECs may also occur. These conditions include multiple endocrine neoplasia disorders (MEN1-3), Von Hippel-Lindau (VHL) syndrome, tuberous sclerosis complex (TS) and neurofibromatosis1 (NF1).
Further information on these inherited disorders can be found at AMEND UK.
Sun exposure – Merkel cell carcinoma (MCC also known as Skin NEC ) is a rare skin cancer that is linked to overexposure to the sun and/or ultraviolet light. Other factors for MCC include advancing age, a weakened immune system, fair skin and /or the Merkel cell polyomavirus (MCV): a common virus that usually lives harmlessly on most people’s skin, but may be a contributory cause for MCC if the virus gets into the merkel cell (neuroendocrine skin cell).
Further information on MCC can be found at Merkel Cell Carcinoma Support.
A family of neuroendocrine cancers.
(Please click on the link for more information about NETs from different locations).
Head & Neck – Pituitary gland – often called PitNETS for short. Thyroid gland – medullary thyroid cancer.
Paragangliomas may also have a neck site of origin.
Less common sites may include larynx, salivary gland, nasopharynx and tonsils.
Lung & Thymic Gland – about 30% of all NENs start in the Lungs.
Gastro-intestinal – NENs of the digestive system. These are the commonest type ( and if pancreas is included, they account for more than 40% of all NETs).
Gi-NENs can occur anywhere in the GI Tract, from the mouth to the anus: but most commonly in the small intestine (Jejeunum and Ileum). (other sites include Oesophageal, Stomach, Appendix, Colon, Rectum).
Hepatobiliary – Pancreatic and Duodenum, Less common primary sites include GB and bile ducts, Liver.
Adrenal gland and Nervous System (ganglia) – also called pheochromocytoma and paraganglioma.
Reproductive and Genito-Urinary Systems – gynaecological structures and organs, breasts, testicles, prostate, bladder and kidneys.
Skin – Merkel cell carcinoma
What are the current treatment options for neuroendocrine cancers (NENs)?
This is dependent on the type and behaviour of your particular neuroendocrine cancer, your overall health, well-being and informed choice.
Although there are a number of treatments, not all will be suitable for all types of Neuroendocrine Cancer – and not all may be licensed or approved for use within the NHS.
Treatments for Neuroendocrine Cancers include:
- Somatostatin analogues (Octreotide or Lanreotide): are hormone injections that can help reduce excess hormone secretion and slow down or stop further cancer growth. Mostly used in Grade 1-2 NETs – but may be used in Grade 3. Available on the NHS.
- Nuclear Medicine whole body therapy – commonly referred to as PRRT (Peptide Receptor Radionuclide Therapy) or Lutathera® : uses irradiated Octreotide (Lutetium-177) to target and treat receptor positive neuroendocrine cancer cells. PRRT using Lutetium-177 is licensed and approved for use, within the NHS, for inoperable or metastatic Grade 1-2 NETs that have primaries within the digestive system (e.g., pancreas or small bowel). In phaeochromocytomas and paragangliomas (PPGLs) – MiBG therapy may be the Nuclear Medicine treatment of choice: named after the radiopharmaceutical used Iodine-131 meta iodobenzylguanidine (I-131 MIBG). It has been used, in the UK, as a treatment for PPGL (and neuroeblastomas in children), since the mid-1980s and has both EMA and (more recently) FDA approval. It is available on the NHS. This type of treatment may also be called RLT (Radioligand Therapy), MRT (Molecular Radionuclide Therapy) OR by radionuclide name, depending on the type of radionuclide and/or neuroendocrine cancer it is being used to treat. PRRT is an industry term.
- Nuclear Medicine liver-targeting therapy – also called SIRT (Selective Internal Radiation Therapy). This therapy takes advantage both of the liver’s dual blood supply (the portal vein and the hepatic artery) and the fact that secondary tumours within the liver mostly gain their blood supply from branches of the hepatic artery. By blocking (embolising) the arterial branches that supply an identified tumour, you can essentially starve it of oxygen and nutrients, while remaining blood supply from portal vein and remaining arterial branches keep surrounding tissue and the rest of the liver healthy. SIRT is a procedure where irradiated micro-beads are injected, through the hepatic artery, to be delivered directly to the tumour – just before embolisation occurs. The irradiated micro-beads get caught in the micro vessels supplying the tumour ensuring that the radiation is concentrated on the cancer cells within it, and the embolisation keeps micro-beads in place, as well as blocking further blood supply to the tumour – leading to cancer cell destruction. There are certain tests and procedures you will have to undergo before SIRT can be safely given. It is not suitable for everyone with neuroendocrine cancer-related liver metastases and is not routinely available, on the NHS, for those with neuroendocrine cancer-related liver metastases. However, SIRT may be available, if you are assessed as clinically suitable, through either a clinical trial (e.g., ArTisaN Study ) or private insurance funding (if available)
- Interventional Radiology (IR) : refers to a range of techniques which rely on the use radiological image guidance to precisely target therapy. These techniques can be used to treat cancer (tumour ablation or embolisation), relieve the effects of the cancer on other systems e.g. blockage of the oesophagus, bowel, kidney (nephrostomy) or liver (biliary drainage), to drain collections of fluid or pus in the chest or abdomen, to place feeding tubes (gastrostomy or jejunostomy) and / or treat collapsed spinal bones (vertebroplasty). Further information on IR in Neuroendocrine Cancer can be found on the Neuroendocrine Cancer UK website here or through watching the short video on general IR, produced by the British Society of Interventional Radiology, here
- Therapeutic endoscopy: is the medical term for a procedure during which treatment is carried out via an endoscope. Therapeutic endoscopy offers an alternative to open surgery for small Neuroendocrine Cancers and/or those that form in polyps. It may also help relieve the effects of the cancer on other systems e.g. blockage of the oesophagus, bowel, or liver (biliary drainage), to drain collections of fluid or pus in the chest or abdomen and/or to place feeding tubes. Treatments include polypectomy, EndoMucosal Resection (EMR), Endoscopic Submucosal Dissection (ESD) – primarily digestive tract NETs (Stomach or Rectal), Laser or BrachyTherapy (for Lung NENs), and Large Loop Excision of the Transformation Zone (LLETZ) – for cervical NENs. Further information on Therapeutic Endoscopy in Neuroendocrine Cancer can be found on the Neuroendocrine Cancer UK website here
- Surgery: In planning treatment, surgery may, in fact, be the first treatment choice considered, as it may offer the only potential chance for cure (in very early stage disease), however, additional consideration is required in regard to possible risks and long-term consequences of surgery. It can also play a significant role in treating metastatic low-moderate Grade NET: where the biology and relatively slow growth rate may allow for debulking (or incomplete resection) of disease to improve symptoms and /or prognosis. Debulking surgery, in selected patients, may also pave the way to allow for other treatments, to be more effective – as tumour burden has been reduced. There are different approaches and aims in surgical treatment – some of these are discussed here.
- Medical anti-cancer therapy: includes chemotherapy, targeted molecular medical therapy, immunotherapy and/or novel medicinal agents.
- Traditional chemotherapy is an anti-cancer treatment where medicine (by infusion or tablet) is used to kill rapidly dividing cells, like cancer cells. There are many different types of chemotherapy, but they all work in a similar way.
- Targeted Molecular Medical Therapy is a type of treatment that uses drugs or other substances to target specific molecules involved in the growth and spread of cancer cells. Blocking these molecules may kill cancer cells or may stop cancer cells from growing or spreading.
In Neuroendocrine Cancer – Platinum-based chemotherapy regimens (e.g., those using Carboplatin or Cisplatin) are primarily used to treat Neuroendocrine Carcinoma, while certain Targeted Molecular Therapies, (e.g., Everolimus and Sunitinib) are more often used in Grade 1-2 Neuroendocrine Tumours.
- Everolimus is currently licensed within the UK to treat inoperable non-functioning, well-differentiated Grade 1-2 NETs that start in the pancreas, gastrointestinal system or lung(s) and are showing signs of ongoing growth and / or spread.
- Sunitinib is currently licensed within the UK to treat inoperable non-functioning, well-differentiated, Grade 1-2 NETs that start in the pancreas and is showing signs of ongoing growth and / or spread.
Combination therapy using Capecitabine and Temozolomide (CapTem / TemCap) is proving to be an effective and relatively safe treatment for patients with advanced well-moderate differentiated NETs of gastroenteropancreatic, lung and unknown primary.
- Immunotherapy uses our immune system to fight cancer, by helping the immune system to recognise and attack cancer cells. Some types of immunotherapy are also called targeted treatments or biological therapies. It can be given on its own or alongside other cancer treatments.
At present the only licensed immunotherapy available on the NHS for Neuroendocrine Cancer – is Avelumab – which is used in the treatment of Merkel Cell Carcinoma (Neuroendocrine carcinoma of the Skin) that has spread (metastasised).
- Surveillance and / or supportive care: Not everyone with Neuroendocrine Cancer will need to be on treatment, and though that may seem strange – it may also be a relief.
- Surveillance can be used to check your cancer and general health for any signs of change that may mean that a treatment might need to be considered.
- Supportive care, (may also be called Symptom control or ‘Palliative Care’) refers to treatment, medications and or procedures or techniques that can be used to alleviate or reduce the impact your cancer, other health issues and /or treatments may be having on you and your physical and mental health. It can include anything from simple medication and / or a combination of some of the treatments mentioned above, to counselling, complementary therapies and practical support. Managing symptoms and ensuring your mental/emotional well-being is an important part of total care and living with neuroendocrine cancer – it is not just for ‘end-of-life’ care.
- Complementary Therapies: not to be confused with “Alternative Therapies”. Complementary therapies in cancer are considered that can be “used alongside conventional cancer treatments”. Further information on Complementary Therapies that may be helpful for those diagnosed with Neuroendocrine Cancer can be found here
What are the current trials for NENs?
There are a number of trials open in the UK which include patients with NENs (as of June 2023) : sources include ClinicalTrials.gov and BePartofResearch databases.
Not all of these trials will be suitable for all types of Neuroendocrine Cancer.
The language used to describe these trials can include a lot of scientific terms. It may help to go through the information before considering participation, to confirm whether you would be eligible to take part – and to ensure you understand the type of trial and what may be involved. If you are invited to join a trial by your medical team, they will explain what these treatments do in more detail.
- A Study of Selpercatinib (LY3527723) in Participants With RET-Mutant Medullary Thyroid Cancer (LIBRETTO-531): Selpercatinib is an inhibitor of the RET (rearranged during transfection) receptor tyrosine kinase. A targeted therapy that stops cancer cells receiving chemical messages that make them grow.
- A Study of Selpercatinib (LOXO-292) in Participants With Advanced Solid Tumors, RET Fusion-Positive Solid Tumors, and Medullary Thyroid Cancer (LIBRETTO-001) (LIBRETTO-001): This is an open-label, first-in-human study designed to evaluate the safety, tolerability, pharmacokinetics (PK) and preliminary anti-tumor activity of selpercatinib (also known as LOXO-292) administered orally to participants with advanced solid tumors, including rearranged during transfection (RET)-fusion-positive solid tumors, medullary thyroid cancer (MTC) and other tumors with RET activation.
- Belzutifan/ MK-6482 for the Treatment of Advanced Pheochromocytoma/ Paraganglioma (PPGL), Pancreatic Neuroendocrine Tumor (pNET), Von Hippel-Lindau (VHL) Disease-Associated Tumors, Advanced Gastrointestinal Stromal Tumor (wt GIST), or Solid Tumors With HIF-2α Related Genetic Alterations (MK-6482-015): This is a study to evaluate the efficacy and safety of belzutifan 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 hypoxia inducible factor-2 alpha (HIF-2α) related genetic alterations.
- A Study to Assess the Pharmacokinetics, Pharmacodynamics, Safety, and Tolerability of Debio 4126 in Participants With Acromegaly or Functioning Gastroenteropancreatic Neuroendocrine Tumors (GEP-NETs) (OXTEND-01): Debio 4126 is sustained-release formulation designed to improve the performance and convenience of somatostatin analogue (SSA) therapy.
- Lutathera and ASTX727 in Neuroendocrine Tumours (LANTana): Patients entered into the study will receive ASTX727 orally up to 3 to 8 days prior to receiving Lutathera treatment to determine whether pre-treatment with ASTX727 results in re-expression of somatostatin receptor-2 in patients with metastatic neuroendocrine tumours.
- Lutetium 177Lu-Edotreotide Versus Best Standard of Care in Well-differentiated Aggressive Grade-2 and Grade-3 GastroEnteroPancreatic NeuroEndocrine Tumors (GEP-NETs) – COMPOSE (COMPOSE): The purpose of the study is to evaluate the efficacy, safety & patient-reported outcomes of peptide receptor radionuclide therapy (PRRT) with 177Lu-Edotreotide as 1st or 2nd line of treatment compared to best standard of care in patients with well-differentiated aggressive grade 2 and grade 3, somatostatin receptor-positive (SSTR+), neuroendocrine tumours of gastroenteric or pancreatic origin.
- Study to Evaluate Safety and Dosimetry of Lutathera in Adolescent Patients With GEP-NETs and PPGLs: This is a multicenter, open-label, single-arm study to evaluate the safety and dosimetry of Lutathera in adolescent patients 12 to <18 years old with somatostatin receptor positive GEP-NETs and PPGLs. The study will enroll at least 8 patients in the GEP-NET cohort and as many adolescents with PPGL as possible in the exploratory PPGL cohort.
- TheraSphere Selective Internal Radiation Therapy (SIRT) as Treatment
for Neuroendocrine Tumours With Liver Mets (ArTisaN): This is an open label study for patients with inoperable metastatic neuroendocrine liver deposits to see whether treatment with Selective Internal Radiation Therapy (TheraSpheres) could lead to improved treatment response rates with acceptable toxicity (minimal serious adverse events reported). This research will also look at the progression free survival and quality of life of the patients who decide to join the study.
Study of Zirconium Zr 89 Crefmirlimab Berdoxam PET/ CT in Subjects With Advanced or Metastatic Malignancies (iPREDICT): The purpose of this study is to evaluate whether zirconium Zr 89 crefmirlimab berdoxam (other names 89Zr-crefmirlimab berdoxam, 89Zr-Df-crefmirlimab, 89Zr-Df-IAB22M2C) PET/CT can predict the response of advanced or metastatic melanoma, Merkel cell carcinoma, renal cell carcinoma, or non-small cell lung cancer tumors to immuno-oncology therapy.
Where can I find neuroendocrine cancer support groups?
Name
Website
Notes
Neuroendocrine cancer UK
A UK-wide, patient co-founded, donation-dependent charitable organisation that provides expert and evidence based information, advice and support for those affected by Neuroendocrine Cancer.
Services include a dedicated helpline, “Natter” support groups – online or in person – for both patients, and friends & family members, online support and discussion forums, 1 to 1 counselling, group therapy programmes, shared experiences, information and education resources – from patient factsheets to videos and podcasts, and information and educational events. NCUK supports research through small grant funding to undertaking or participating in research projects and initiatives with charity, patient community and clinical partnership working.
NCUK also works with AMEND to provide information and support for those affected by adrenocortical cancer (ACC): ACC Support UK
A site by Ronny Allan – Living with neuroendocrine cancer
A site and award-winning blog by Ronny Allan – Living with neuroendocrine cancer
A very high profile blogger and patient advocate diagnosed with Metastatic Neuroendocrine Tumours (Small Intestine NET) in 2010.
Ronny’s 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.
AMEND
A UK patient-founded charity, that provides expert and evidence based information, advice and support for those affected by multiple endocrine neoplasia (MEN) syndromes types 1, 2, and 3, and SDH syndromes. These inherited syndromes carry an increased risk for the development of neuroendocrine cancers. AMEND also provides support to those with medullary thyroid cancer (MTC) and phaeochromocytomas and paragangliomas (PPGLs).
AMEND also runs two other groups:
ACC Support UK (for those affected by adrenocortical cancer – in collaboration with Neuroendocrine Cancer UK)
Phaeo Para Support UK (for those affected by inherited phaeochromocytoma and paraganglioma syndromes)
Services include helpline, support groups, 1 to 1 counselling, shared experiences, information and education resources – from patient factsheets to videos and podcasts, and information and educational events. AMEND wishes to support research and is provides grants when funds allow to enable medical researchers to supplement an existing project or pump-prime new research ideas.
NETRF
A patient founded charitable organisation based in America, committed to improving the lives of patients, families, and caregivers affected by neuroendocrine cancer by providing information and educational resources.
INCA
The International Neuroendocrine Cancer Alliance is the global voice in support of patients with neuroendocrine cancers (NENs) and genetic syndromes (GenNETs). INCA is an umbrella organisation representing 33 patient advocacy and research groups from around the world.
A key priority in INCA’s work is creating common platforms and working in collaboration with medical communities to fulfil the informational needs of patients, ensure access to the highest standards of care and facilitate patient involvement in research. The website is available in a multitude of languages.
Catherine Bouvier-Ellis, co-founder and CEO of Neuroendocrine Cancer UK, is a former president of INCA – and the charity is a core member.
Awareness and symbols
World NET awareness day is held on 10th November. It is coordinated by the International Neuroendocrine Alliance.
The ribbon for NET cancers is a zebra print.
Image from Wikimedia Creative Commons.
Why a zebra?
Try this thought experiment. You hear hoofbeats coming towards you – what could it be? It’s reasonable to assume it’s a horse. Horses are very common in most places. It’s highly unlikely that the source of the sound is a zebra.
But just occasionally, it really is a zebra.
This is often the situation with NETs. Rare, unexpected and something doctors need to consider when dealing with symptoms that may point to other causes.
