All that is to say that, we, like all cancer-touched families, were devastated. A week later, his cancer was confirmed. Grief quickly turned to relief, however, when, after testing, he was given “good” news: He had a pancreatic neuroendocrine tumor (“PNET” for short), a rarer form of pancreatic cancer that accounts for just 7 percent of total pancreatic cancers, according to PanCAN. The five-year survival rate for PNETs is remarkably more optimistic: If localized — meaning it has not spread — it’s as high as 93 percent, according to the American Cancer Society. (Sadly, Steve Jobs, who died in 2011, had the same type of tumor, but opted to forgo treatment until it had become too advanced to treat easily, notes Harvard Health.) “The doctors say they can remove it with surgery, and I won’t need chemo or radiation,” my dad told us. Excuse me, what? RELATED: 2 Challenges and 3 Advances in Treating Pancreatic Cancer
PNET: The ‘Good’ Kind of Pancreatic Cancer
A PNET differs from what we think of as “classic” pancreatic cancer. The majority of pancreatic cancers are adenocarcinomas that grow in ductal cells in the pancreas (which secrete juices that aid digestion), notes PanCAN. They are known for being swift to grow and spread, and are often unable to be removed through surgery, as they typically are only detected after they have spread. PNETs, on the other hand, like my dad had, grow in islet cells in the pancreas, which make hormones that control your blood sugar, PanCAN explains. These are slower to grow and are well-defined (as opposed to being “diffuse,” or spread out, like adenocarcinomas), meaning surgery to remove them is frequently possible. Many PNETs are only found “incidentally,” like if someone receives scans following a car accident, Xavier M. Keutgen, MD, director of the neuroendocrine tumor program at the University of Chicago, told me on the phone in an interview for this article. (Dr. Keutgen did not treat my father.) Perhaps that’s why my dad’s case was so remarkable. Last July, he was dealing with some weird digestive issues. Despite having a normal appetite and not changing his diet at all, he lost 10 pounds in an alarming number of weeks. That’s what got him to the doctor in the first place. His doctors suspected irritable bowel syndrome (as a man in his sixties, he would have been an atypical patient), but sent him for a CT (computerized tomography) scan “just to rule out the bad stuff.” A PNET is a sight to see on a scan, says Keutgen. “On a CT scan with IV [intravenous] contrast, a PNET commonly lights up bright because they have a lot of vasculature and therefore take up the contrast agent very well. They also usually appear very well circumscribed on CT and MRI [magnetic resonance imaging]. These tumors look like a bright, well-defined marble sitting in the pancreas.” A scan alone can be used to detect a PNET, but they can be biopsied with the help of an endoscopic ultrasound, which will determine the final diagnosis. Unfortunately — or maybe fortunately, depending on how you look at it — my dad’s CT scan revealed that telltale marble sitting on his pancreas. RELATED: FDA Approves New Treatments for Some Types of Neuroendocrine Tumors
Surgery Is the Best Hope for a PNET Cure
My dad’s situation was both straightforward, and yet not. Guidelines recommend that PNETs that measure less than 1 centimeter (cm) and are not causing any symptoms be left alone, as these types of tumors are slow-growing, says Keutgen. On the other hand, people that have tumors that are larger than 2 cm should have them surgically removed, if possible. This depends on the patient’s health, the location of the tumor in the pancreas, and if lymph nodes are affected. Treatment for patients whose tumors measure between 1 and 2 cm is more controversial and can include surgical resection (removal of cancerous tissue) or observation. Keutgen adds that a myriad of factors are at play in this situation and it is very important that an experience clinician has an honest discussion with the patient about which option is best indicated or preferred in this situation. “If the tumor is localized to the pancreas and hasn’t spread to [other organs like] the liver (the main source of concern, as far as metastasis), surgery is the only cure,” says Keutgen. My father’s tumor fit the bill for surgery. Follow-up scans indicated that his cancer hadn’t spread beyond the pancreas. He’d still need a portion of his pancreas removed, however. And there were a lot of unknowns. The tumor was wrapped around a vein that supplied 70 percent of the blood to his liver, for example, and they’d have to get a vascular surgeon in to remove a portion of a vein from his leg to use for reconstruction. It would be a long and involved surgery. Luckily, he lives in an area near a hospital with a surgeon who specializes in PNET removals, which is not common. RELATED: Recovering After Pancreatic Cancer Surgery: 5 Top Tips
There Are Other Options for PNET Treatment
If my dad couldn’t get surgery or his PNET had metastasized, he would have had other options, says Keutgen. And advances in treatment are rather exciting. If the tumor has spread to the liver, the main tumor in the pancreas can be removed, and some medical centers, like UChicago, use a surgical techniques called parenchymal-sparing neuroendocrine tumor resection to clean cancer cells out of the liver while sparing as much of the organ as possible. “Eventually, tumors will come back in the liver, but they’re so slow-growing. You gain 5 to 10 years or more because we believe this technique resets the time clock on the cancer,” he says. If surgery wasn’t an option at all, hormonal injections or oral chemotherapy pills can be used to slow down tumor growth, sometimes for years. Then, there’s peptide receptor radionuclide therapy, or PRRT. “This is a new targeted therapy. It’s an infusion with radioactivity that patients receive every other month for four cycles (a total of eight months),” explains Keutgen. “It targets only NET cells by docking on these cells and putting radioactivity in them,” he says. PRRT is usually not curative but it can shrink the tumor in one-third to one-half of patients and slows its growth in a majority of them, says Keutgen. There are also few side effects (mainly some fatigue), which makes it an attractive option. Keutgen’s team is currently researching how to improve tumor response rate of PRRT in his laboratory at UChicago. “By using therapies like surgery and PRRT for tumors that have spread to the liver and other organs, we allow patients to live a long time. Our goal is that patients live a normal life span with a great quality of life and eventually die with the tumor, not of the tumor,” says Keutgen. RELATED: How to Eat When You Have Pancreatic Cancer
Our Story: A Happy Ending
As for my dad’s surgery, it was successful. It was nine hours and involved the transfusion of a lot of blood. The lymph nodes came back clean — a strong sign that his cancer hadn’t spread. His recovery was long and scary, and he’s still learning what it means to manage a new normal with a (partial) pancreas that doesn’t function like it used to. He needs to take pancreatic enzymes before every meal, which are incredibly expensive. He has a form of diabetes because his pancreas doesn’t make as much insulin as it should, so he has to closely watch the carbohydrates in his diet and give himself daily insulin injections. He has always loved to cook, but for him, living as a cancer survivor means spending his time making elaborate — and incredibly healthy — meals. (It’s a form of stress management and self-care, and helps him exert a bit of control over his situation.) My dad gets a CT scan every three months to make sure the cancer hasn’t returned or doesn’t appear in his liver. And he gets lab tests to measure his blood cells, which check for signs of cancer. It’s only been one round of checks, thus far, but so far, so good. “As far as I’m concerned, you no longer have cancer,” my dad’s doctor told him. And you know what? He looks healthier and happier than ever. RELATED: After Pancreatic Surgery: Know Your Risk for EPI
