Innovative therapy improves lives of people with neuroendocrine tumors

When Jay Elsten's neuroendocrine tumors no longer were responding to traditional treatment, Mayo Clinic offered him a recently approved nuclear medicine therapy. And it worked. Within a week, Jay's symptoms were gone.

Jay Elsten's medical concerns started subtly with just some itching and rashes on his legs. A visit to his primary care physician in 2012 led to a diagnosis of hepatitis, a prescription for antibiotics and a recommendation to call again if Jay didn't feel better in a few days.

"In three days, I couldn't keep anything down," Jay says. "I'd lost 40 pounds in a week, and (my doctor) said to go to the ER."

That hospital visit led to more tests, including a CT scan. Results showed a tumor on Jay's pancreas. At that point, Jay, who lives in Missouri, had to decide where to go next.

"It was too rare, what I had. (My doctor) gave me three choices," Jay says. "The business I was in before, I had dealings with people in Dodge Center, Minnesota, so I knew people up here. I thought, 'I'll go to Mayo because if I get in trouble, I know people up there that can help my wife.'"

An unusual diagnosis

When he arrived at Mayo Clinic in Rochester, a biopsy revealed that Jay had a neuroendocrine tumor on his pancreas. "Neuroendocrine tumors are really a collection of tumors that can arise anywhere in the body," says Thorvardur Halfdanarson, M.D., a Mayo Clinic medical oncologist. "And not only can they arise anywhere in the body, but they behave very differently from one person to the next."

For example, some people experience diarrhea or pain as a result of these tumors, while others have no symptoms, and the tumors are discovered only during an imaging exam for something else. Neuroendocrine tumors can secrete hormones, which often triggers symptoms. Some neuroendocrine tumors are slow-growing. Others are aggressive.

"They are still a big enigma, and for the most common ones — the small bowel neuroendocrine tumors — we really don't have a good understanding why people get them," Dr. Halfdanarson says.

Once he learned his diagnosis, Jay knew what he needed to do. "I was here to fight it from the beginning, and it's what I intend on doing. I want to see my kids graduate. I want to walk my daughter down the aisle. I intend on staying around for a long time," says Jay, who is married and has two children.

A tough battle

A Mayo Clinic care team that included specialists from Medical Oncology, Gastroenterology, Pulmonary Medicine, Surgery, Radiology, Nuclear Medicine and Pathology has supported Jay throughout his treatment.

"The care plan never changes. Where other places, you get a new doctor, and they've got a different idea," Jay says. "They decided from the beginning what they were going to do, and that's the same plan that we've carried out through the whole seven years."

The plan started with chemotherapy. That shrank the cancer and gave Jay two years without any issues. But then the cancer spread to his liver. Chemotherapy did not effectively bring his neuroendocrine tumors in check at that point, and his team looked for other options.

In January 2018, the treatment Jay needed was approved by the Food and Drug Administration to treat the type of tumor he had: gastroenteropancreatic neuroendocrine tumors. That treatment is called lutetium Lu 177 dotatate, a peptide receptor radionuclide therapy, also referred to as PRRT.

"It's kind of a Trojan horse," says Geoffrey Johnson, M.D., Ph.D., chair of Mayo Clinic's Division of Nuclear Medicine, adding that the dotatate binds to receptors on the cancer cells and the lutetium radiates the cells, killing them.

"PRRT … is a therapy using a radioactive compound that sticks to the neuroendocrine tumor cells. There are three components to it. There is what's called a somatostatin analog, which binds to the receptors of the tumor cells. And then there is therapeutic radionuclide, or radioactive molecule, which is therapeutic. And then there is almost like a glue that binds these things together," Dr. Halfdanarson says. "We inject this into a vein that circulates around the body. It sticks to the tumor cells that express the somatostatin receptors. Then this radiation molecule sits right on the tumor cell and kills the tumors cells with radiation."

Positron emission tomography imaging, with gallium Ga 68 dotatate, identified Jay as an ideal candidate for the new treatment. With this treatment, patients receive four doses, each eight weeks apart, with checkups about a month after each infusion. For most people, symptom improvement comes after the second dose.

A remarkable result

Jay says that being able to take advantage of this kind of innovative therapy is part of the advantage of the long-term care he's received at Mayo Clinic. "The longer I can push this disease off, things change," he says. "(Lutetium Lu 177 dotatate) is something that didn't exist seven years ago when I was first diagnosed."

His care team tried to set Jay's expectations appropriately. "Dr. Halfdanarson told me before I got the first treatment: 'Don't expect immediate results. It usually takes two rounds before we see anything,'" Jay says.

But he was in for a surprise. "Within three or four days, the diarrhea stopped," Jay says. "It had shocked the tumor enough … it quit producing that hormone. Within a week, I was back to normal. It was simply amazing."

Rachel Eiring, a medical oncology physician assistant, was part of Jay's Mayo Clinic care team during his lutetium Lu 177 dotatate treatments, and she was thrilled with his results.

"I think what's absolutely remarkable about his story is the huge improvement in his quality of life," she says. "He went from … being in and out of the hospital to being able to do the things that he wanted. That was exciting to see somebody have such a big impact following just his first treatment."

"It's been a great experience up here," Jay says. "When I was in the hospital in Joplin (Missouri) with all this, there was a patient in there, and one of the nurses asked me if I would go talk to him about Mayo. I'm happy to talk to anybody about Mayo. My family knows it. They probably get tired of hearing about it. But it's just an amazing place."

Watch this video to learn more about Jay's story:

This story originally appeared on the Sharing Mayo Clinic blog.

Learn more about neuroendocrine tumors.

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