Pancreatic neuroendocrine tumours (PNETs) — a different disease, explained plainly

If your report says neuroendocrine, most of what you have already read about pancreatic cancer was about a different disease. A pancreatic neuroendocrine tumour starts in a different cell, grows at a different speed, is treated on a different pathway and carries a different outlook. The two are grouped together only because they begin in the same organ.

The pancreas does two separate jobs. Most of it makes digestive juice, which drains through a system of ducts into the small bowel. Scattered through that tissue are small clusters of hormone-making cells, the islets, which release insulin, glucagon and other hormones straight into the bloodstream. Pancreatic ductal adenocarcinoma begins in the duct lining. A pancreatic neuroendocrine tumour, usually shortened to PNET or written as pNET, begins in those hormone-making cells. Older reports and older textbooks call the same thing an islet cell tumour, which is the phrase many families are handed first.

That difference is not a technicality. PNETs are uncommon, they often grow slowly over years rather than months, many are found by accident on a scan done for something else, and a good number are still operable when they are found. Their outlook, as a group, is considerably better than that of ductal adenocarcinoma. PNET versus adenocarcinoma — the differences that matter sets the two side by side, and the complete pancreatic cancer guide covers the far more common ductal type.

This page is the starting point for the neuroendocrine side. It explains what the diagnosis means, the two questions that shape everything that follows — whether the tumour makes a hormone, and how fast its cells are dividing — and where each part of the pathway is delivered. Read it once, slowly. Very little about a PNET needs deciding this week.

• Does it say neuroendocrine tumour or neuroendocrine carcinoma? Those two phrases describe very different diseases and lead to very different plans. Ask for the exact wording rather than a summary of it.
• What is the grade, and what is the proliferation index? This is what separates a slow tumour from a fast one. PNET grade and the proliferation index explains what the pathologist is measuring.
• Is it functioning or non-functioning? If a hormone is being released, controlling that effect can matter more urgently than the tumour itself. Functioning versus non-functioning PNETs sets out the difference.
• Has anything shown up outside the pancreas? Spread to the liver does not carry the same meaning here that it carries in adenocarcinoma, and it does not automatically rule out surgery or a long course of treatment.
• Is a somatostatin receptor scan planned? A DOTATATE PET shows where receptor-rich tumour tissue sits, and also indicates whether receptor-targeted treatment could work later.
• Should anyone in my family be assessed? Worth asking if you were diagnosed young, have more than one tumour, or have relatives with endocrine tumours. Genetic counselling is available in-house at CION.
• What are my options, and does anything need deciding this week? For many PNETs the honest answer is no. How pancreatic neuroendocrine tumours are treated lays out the choices before you have to make one.

1. 1

   Dedicated imaging of the pancreas

   A pancreatic-protocol contrast CT, or an MRI where that answers the question better, maps the tumour, its relationship to the vessels behind the gland, and the liver.

   Ordered and reported in-house at CION
2. 2

   Bloods, and hormone tests if the picture fits

   Routine bloods and neuroendocrine markers are taken. Where symptoms suggest a functioning tumour, the specific hormone is measured under the right conditions rather than at random.

   In-house at CION
3. 3

   Tissue, usually through endoscopic ultrasound

   A fine-needle sample taken through an endoscope confirms that the tumour is neuroendocrine and gives the grade. Almost nothing can be decided properly without it.

   Coordinated with specialist endoscopy partners
4. 4

   A receptor scan where it will change something

   A DOTATATE PET shows which deposits are receptor-rich. It answers where the disease is, and whether receptor-targeted treatment is an option worth holding in reserve.

   Coordinated with partner nuclear medicine centres
5. 5

   The case is reviewed as a team

   Imaging, pathology, hormone results and your general health are discussed together, so that medical oncology, radiation oncology and the surgical partners agree one plan rather than three.

   Tumour board at CION
6. 6

   A plan, with the review points written down

   Monitoring, surgery or systemic treatment is chosen with the reassessment dates agreed in advance. How pancreatic neuroendocrine tumours are treated explains each route in full.

   Free 45-minute consultation

A neuroendocrine pathway is delivered by more than one team, and it is better to know that at the start than to discover it with an invoice. Your first consultation with us is free and lasts 45 minutes. It is a genuine review of your reports, not a booking appointment.

Delivered in-house at CION, across 35+ centres in Telangana and Andhra Pradesh: the systemic treatment of neuroendocrine tumours, including somatostatin-analogue-class therapy to slow growth and settle hormone symptoms, targeted oral treatment of the mTOR-inhibitor and TKI classes, and chemotherapy where the grade calls for it. Also radiation where it is appropriate; the ordering and reporting of pancreatic-protocol CT, MRI and bloods; genetic counselling for inherited syndromes; nutrition and pancreatic enzyme support; pain relief, psycho-oncology and supportive care; and long-term follow-up. Pancreatic cancer treatment in Hyderabad sets out how those services are organised across the network.

Coordinated with specialist HPB, gastroenterology, endoscopy and nuclear medicine partner centres, and may be billed there: all pancreatic surgery, from removing a small insulinoma to the larger resections; endoscopic ultrasound with biopsy; ERCP and biliary or duodenal stenting; staging laparoscopy; coeliac plexus block for pain; PET-CT and DOTATATE PET; and peptide receptor radionuclide therapy, which is given at a partner nuclear medicine unit. We arrange these, we sit in on the decisions, and we tell you in advance where each one happens and who invoices you. We do not describe them as our own theatre, endoscopy or radionuclide lists, because they are not.

This is still a cancer, and some PNETs are aggressive. Saying otherwise would be dishonest, and you would spot it. But the picture is genuinely different from the one the phrase “pancreatic cancer” brings to mind. Many PNETs grow slowly enough that a small, quiet, low-grade tumour can reasonably be watched rather than removed. Many are operable when found. Even when a PNET has spread to the liver, people often live well for a long time on treatment that controls it, which is not how liver spread from adenocarcinoma usually behaves.

Because the disease often moves slowly, the pace of care can be slower too. There is usually time to get the pathology right, to obtain a receptor scan, to seek a second opinion and to think. Decisions made carefully over the first month tend to serve people better than decisions made in the first week. If you are being pushed to start something immediately without a confirmed grade, that is worth questioning out loud.

The survival figures you will find online are a poor guide here. Most of them pool very different situations together, and some pool neuroendocrine tumours with ductal adenocarcinoma, which drags the picture down for no good reason. PNET prognosis and survival explains what genuinely shifts the outlook — grade, extent of disease, whether surgery is possible and how the tumour answers treatment — without pretending that a group average describes one person.

Medical disclaimer: This page explains what a pancreatic neuroendocrine tumour is and how the pathway is organised, and is reviewed by a CION medical oncologist with reference to the WHO classification of neuroendocrine neoplasms and NCCN guidance on neuroendocrine tumours. It is general information and deliberately states no survival figure, because no published figure describes an individual; your own plan depends on the grade, hormone status, extent of disease and your general health, and must be decided with your treating team. Systemic therapy for neuroendocrine tumours including somatostatin-analogue-class treatment, targeted oral therapy and chemotherapy, radiation where appropriate, the ordering and reporting of CT, MRI and blood tests, genetic counselling, nutrition and pancreatic enzyme support, pain relief, psycho-oncology and long-term follow-up are delivered by CION; all pancreatic surgery, endoscopic ultrasound and biopsy, ERCP and biliary or duodenal stenting, staging laparoscopy, coeliac plexus block, PET-CT and DOTATATE PET, and peptide receptor radionuclide therapy are coordinated with specialist hepatobiliary, gastroenterology, endoscopy and nuclear medicine partner centres and may be billed there.