Pancreatic Neuroendocrine Tumor: Your Doctor Explains

It often starts subtly. Maybe it’s a nagging ache in your belly, or you’re just feeling wiped out all the time, more than usual. You might notice your digestion is off, or strange swings in your blood sugar. Then, after some visits and tests, you hear the words: Pancreatic Neuroendocrine Tumor. It’s a mouthful, isn’t it? And hearing it can feel like the world just tilted on its axis. These growths, sometimes called PNETs or islet cell tumors, are pretty rare. They start in the special hormone-making cells of your pancreas. It’s a lot to take in, I know. But we’re here to walk through what this means, step by step.

Table of Contents

What Exactly Is a Pancreatic Neuroendocrine Tumor?

So, let’s break it down. Your pancreas – it’s a small, flat organ, kind of shaped like a pear, tucked away behind your stomach. It does some really important jobs. Part of it helps with digestion, and another part has endocrine cells. These are the cells that make hormones, which are like little messengers that control things like your blood sugar.

A Pancreatic Neuroendocrine Tumor (PNET) is a tumor that grows from these endocrine cells in your pancreas. Now, “tumor” can be a scary word, but it’s important to know they can be either benign (meaning not cancerous and won’t spread) or malignant (cancerous, with the potential to spread if not treated). Our main goal is always to figure out which kind it is and the best way to manage it.

Different Kinds of PNETs

It turns out, there isn’t just one type of PNET. They’re often named after the hormone they make too much of, or the cells they start in. Here are some of the main ones we see:

Insulinomas: These are the most common “functioning” PNETs (meaning they make extra hormones). They produce too much insulin, which can make your blood sugar drop too low. Good news is, most of these (about 90%) are not cancerous.
Gastrinomas: These make too much gastrin, a hormone that tells your stomach to produce acid. This can lead to severe ulcers. A little over half of these can be cancerous.
Glucagonomas: These affect cells that make glucagon, a hormone that raises your blood sugar. These are often cancerous.
VIPomas: “VIP” stands for “vasoactive intestinal peptide.” This hormone affects water and salt balance in your gut and relaxes digestive muscles. VIPomas can cause a lot of watery diarrhea and can be cancerous.
Somatostatinomas: These are usually cancerous but tend to grow slowly. They make too much somatostatin, a hormone that juggles several body functions.

Some PNETs are “non-functioning,” meaning they don’t release extra hormones that cause obvious symptoms. These are often found when they grow large enough to press on other organs, or sometimes by chance during a scan for something else.

What Might You Notice? Signs and Symptoms

Because PNETs can make different hormones, the symptoms can really vary. And sometimes, especially with non-functioning tumors, there might not be many symptoms at first. But here’s what we often hear about:

That awful acid reflux that just won’t quit.
Persistent diarrhea.
Feeling incredibly tired (fatigue).
Signs of blood sugar swings: being super thirsty, peeing a lot more.
Indigestion or an upset stomach.
Losing your appetite or feeling full quickly.
Nausea and sometimes vomiting.
Unexplained weight loss.
For some types, you might experience carcinoid syndrome – this can include uncomfortable flushing of your face and neck, and sometimes wheezing.
Big swings in your blood sugar, either too high or too low, depending on the tumor type.

What Causes These Tumors?

Honestly? We’re still figuring out the exact ‘why’ for most PNETs. It’s not usually something you did or didn’t do.

For a small number of people, PNETs can be linked to inherited genetic conditions, like Multiple Endocrine Neoplasia type 1 (MEN1). If you have MEN1, you have a higher chance of developing tumors in various endocrine glands, including the pancreas. That’s why we’ll always ask about your family’s health history.

Potential Complications

The biggest worry, especially with cancerous PNETs, is that they can metastasize, which is the medical term for spread. If they do spread, they often go to places like your liver, bones, or lungs. Catching them early is key.

How We Figure Out What’s Going On: Diagnosis and Tests

If you’re having symptoms that make us think about a PNET, or if one is suspected for other reasons, we’ll start with a thorough chat. I’ll ask about your symptoms – when they started, what makes them better or worse. We’ll do a physical examination. And, as I mentioned, we’ll talk about your family history.

Then, we’ll likely suggest some tests to get a clearer picture:

Blood tests: We can check your general health and look for specific hormone levels. If certain hormones are very high, it can point us towards a functioning tumor.
Abdominal Computed Tomography (CT) scan: This uses X-rays and a computer to create detailed pictures of your insides, including your pancreas.
Magnetic Resonance Imaging (MRI): This uses magnets and radio waves for even more detailed images, especially good for soft tissues.
Endoscopic Ultrasound (EUS): This is a neat test. A thin, flexible tube with a tiny ultrasound probe on the end is passed down your throat into your stomach and the first part of your small intestine. This lets us get very close-up ultrasound pictures of your pancreas.
Endoscopic Retrograde Cholangiopancreatography (ERCP): Similar to an EUS, but it can also be used to look at the ducts (tubes) in and around your pancreas and liver.
Neuroendocrine Positron Emission Tomography (PET) scan: This is a special type of scan that’s very good at finding neuroendocrine tumors. You’ll get an injection of a slightly radioactive substance that these tumor cells tend to pick up, making them light up on the scan. Common types are Gallium-68 DOTATATE PET scans.

Sometimes, to be absolutely sure, we need a small piece of the tumor. This is done with a needle biopsy, often guided by an ultrasound or CT scan. An interventional radiologist, a doctor who specializes in these image-guided procedures, might do this. The sample then goes to a pathologist – that’s a doctor who’s an expert at looking at cells under a microscope – to check for cancer cells and get more details about the tumor.

All these tests help us understand if the tumor is functioning (making hormones) or non-functioning, and what specific type it might be.

Understanding Your Pancreatic Neuroendocrine Tumor: Staging and Grading

If a PNET is found to be cancerous, the next step is to determine its stage and grade. This sounds technical, but it just helps us understand how much the cancer has grown and how quickly it might be behaving.

Cancer staging tells us about the tumor’s size and if it has spread:

Stage I: The tumor is small (less than 2 cm, about ¾ inch) and hasn’t spread outside the pancreas.
Stage II: It’s a bit bigger (more than 2 cm) or might have started to grow into nearby areas like the first part of your small intestine (duodenum) or your bile ducts.
Stage III: The tumor has spread to nearby lymph nodes, or into nearby organs or large blood vessels.
Stage IV: The cancer has spread to distant parts of your body, like your liver or bones.

Cancer grading tells us how the cells look under the microscope and how fast they seem to be dividing:

Grade 1 (Well-differentiated): The cells look quite a bit like normal cells and aren’t multiplying very fast. These tend to be slower-growing.
Grade 2 (Well-differentiated): The cells are somewhere in between Grade 1 and Grade 3 in terms of how they look and how fast they’re growing.
Grade 3 (Well-differentiated or Poorly differentiated):
Well-differentiated Grade 3 NET: Cells are multiplying more quickly, and look more abnormal, but still have some neuroendocrine features.
Poorly differentiated Neuroendocrine Carcinoma (NEC): The cells are dividing very rapidly and look very abnormal. These are more aggressive. Sometimes called small cell or large cell neuroendocrine carcinoma.

This information is crucial for planning the best treatment for you.

How We Treat Pancreatic Neuroendocrine Tumors

Alright, so what can we do? Treatment really depends on your specific situation – the type of PNET, its stage and grade, whether it’s functioning or non-functioning, and your overall health.

For many early-stage PNETs, both cancerous and noncancerous, surgery to remove the tumor (pancreatectomy) is often the main treatment and can sometimes be a cure. The type of surgery depends on where the tumor is in your pancreas. You might hear your surgeon talk about the “head,” “body,” or “tail” of the pancreas – it’s just describing different parts of the organ.

Common surgeries include:

Whipple procedure (pancreaticoduodenectomy): This is a major operation, often used if the tumor is in the head of the pancreas. The surgeon removes the head of the pancreas, the duodenum, the gallbladder, and part of the bile duct.
Central pancreatectomy: If the tumor is in the “neck” or “body” (the middle part) of the pancreas, the surgeon might remove just that central section, trying to preserve the head and tail.
Distal pancreatectomy: This is for tumors in the tail (the narrow end) of the pancreas. The surgeon removes the tail, and sometimes part of the body of the pancreas, and often the spleen as well, because it’s very close by.

If surgery isn’t an option, perhaps because the PNET has spread or it would be too risky, there are other effective treatments your oncologist (cancer specialist) might discuss:

Hormone therapy (Somatostatin analogs): These drugs can help control symptoms from functioning tumors and can also slow down the growth of some PNETs.
Peptide Receptor Radionuclide Therapy (PRRT): This is a clever treatment where a radioactive substance is attached to a molecule that targets PNET cells. It delivers radiation directly to the tumor cells, wherever they are in the body.
Targeted therapies (like Tyrosine Kinase Inhibitors or mTOR inhibitors): These are newer drugs that target specific pathways or proteins involved in cancer cell growth.
Chemotherapy: This uses strong medicines to kill cancer cells or stop them from growing. It’s used more often for higher-grade or more aggressive PNETs/NECs.

If the PNET has spread to your liver, there are also specific treatments that can target the liver tumors, like ablation (destroying tumors with heat or cold) or embolization (blocking blood supply to the tumors).

What About Recovery?

Recovery really depends on the treatment you have. Surgery, especially a big operation like the Whipple procedure, takes time. You might be in the hospital for about a week, and it could take a good 8 to 12 weeks before you’re feeling back to your usual activities. We’ll be with you every step of the way, managing pain and helping you get back on your feet.

Keeping in Touch With Your Care Team

It’s so important to let us know if anything changes. If your original symptoms come back or get worse, or if you notice anything new that’s worrying you, please don’t hesitate to reach out. It might be nothing, or it might be something we need to check. The best way to know is to talk to your doctor or your specialist team.

Looking Ahead: What’s the Outlook?

This is often the first question on people’s minds, and it’s a tough one because everyone’s journey is unique. When we talk about survival rates, we’re looking at statistics – for example, the percentage of people who are alive five years after their diagnosis. The American Cancer Society provides these general figures for PNETs:

If the cancer is localized (only in the pancreas): The 5-year relative survival rate is about 96%.
If it’s spread regionally (to nearby structures or lymph nodes): The 5-year relative survival rate is about 77%.
If it’s spread to distant parts of the body: The 5-year relative survival rate is about 27%.

It’s really important to remember that these are just averages. Your specific type of PNET, its grade, the stage at diagnosis, your age, your overall health, and how well the treatment works – all these things play a big role. Many people with PNETs, especially those with slower-growing types or those caught early, live for many years, and can have a good quality of life. Some PNETs, even if they’ve spread, can be managed as a chronic condition for a long time.

Your oncologist and surgeon are the best people to talk to about what these numbers might mean for you. They know your individual situation.

Key Takeaways About Your Pancreatic Neuroendocrine Tumor Journey

This is a lot of information, I know. If I could boil it down to a few key things to remember about a Pancreatic Neuroendocrine Tumor, it would be this:

PNETs are rare tumors that start in the hormone-making cells of your pancreas.
They can be cancerous or non-cancerous, and symptoms vary widely depending on if they make excess hormones.
Diagnosis involves several tests, including imaging and sometimes a biopsy.
Treatment depends on many factors but can include surgery, hormone therapy, PRRT, targeted therapy, or chemotherapy.
The outlook is very individual, and many people live well for a long time with PNETs.

You’re Not Alone in This

Hearing you have a Pancreatic Neuroendocrine Tumor can be overwhelming, there’s no doubt about it. But please know, you’re not alone. Your medical team is here to support you, to answer your questions (no matter how small they seem), and to help you navigate every step of this. We’ll face it together.