When Richard went to the emergency room in mid-2016, his symptoms were familiar — extreme stomach pain, digestive issues and just generally not feeling well.
He had spoken to his primary care doctor multiple times over the previous three years about the terrible pain and indigestion. The doctor prescribed medications for acid reflux and muscle relaxants and even ordered a colonoscopy, but it didn’t find anything out of the ordinary.
"You start feeling like you’re going crazy, because you know that there is something wrong, but the tests aren’t showing anything," he says.
Diagnosis of NETs Provides Answers and More Questions
Richard changed his diet to eat mostly bland foods, which helped. But on a vacation with his wife in 2016, the new foods caused his symptoms to flare up. When he got home, he was in such extreme pain that he went to the ER. A nurse practitioner, concerned that the symptoms didn’t seem like normal acid reflux, ordered a CT scan.
"They found tumors in my pancreas and liver, and that’s when they discovered I potentially had cancer," says Richard. He met with an oncologist at a nearby regional hospital, where they did a biopsy and diagnosed him with neuroendocrine tumors, or NET.
There are many types of NETs that can begin in any part of the body. The most common sites for these tumors are the gastrointestinal (GI) tract, lungs, or pancreas. Tumors can also form in the adrenal glands, or hormone-producing areas like the thyroid or pituitary gland. About 100,000 people in the U.S. have NETs.
Richard was diagnosed with stage 4 pancreatic neuroendocrine tumor that also spread to his liver. "It’s a tough disease to diagnose because the symptoms mimic so many other things," he says. "I look back at it now and I think what I had were classic neuroendocrine cancer symptoms. But a lot of doctors aren’t specialists in that."
Despite learning that he had an incurable cancer, Richard did feel some relief when he learned that even at stage 4, a pancreatic neuroendocrine tumor is quite treatable.
Finding a NETs Specialist
Richard and his wife Peggy started researching the condition. His original oncologist said Richard wasn’t a candidate for surgery and suggested transarterial chemoembolization (TACE). In TACE, chemotherapy drugs are injected directly into the blood vessel leading to a tumor. Then, doctors use a material called an embolic agent to block the artery, trapping the anti-cancer drugs inside, close to the tumor.
TACE is often used for GI cancers that spread to the liver. It can provide good results for patients with few other treatment options. But Richard wasn’t happy being told he only had one choice.
"I was a little disheartened thinking, 'Wow, that’s the only treatment available?'" he says. He and Peggy had already decided that they were willing to go anywhere in the country to get the best neuroendocrine tumor treatment.
The Penn Medicine Difference
Richard and Peggy kept looking and found Penn Medicine’s extensive NET program. He scheduled an appointment for a second opinion.
"When I went to Penn, it was completely different," Richard says. "They didn’t really rule anything out, including surgery. All these treatments were on the table." His Penn doctors, beginning with Paul Wissel, MD, laid out a plan with a sequence of treatments that left options for the future.
"Boy, that was just an eye-opener for me," Richard says. "To hear that I’m not being excluded initially from other treatments. They were really finding the right specific treatment for me."
Even the way the doctors and other staff members talked to Richard was different, he says. The discussions were forward-looking, planning for a future in ways his previous doctors had not. "They talked about upcoming treatments that were in the pipeline and clinical trials. For the first time, I was able to see a future living with the disease."
The Back-and-Forth Battle with NETs
In September 2016, a month after his first appointment, Penn Medicine doctors started him on lanreotide injections. This drug inhibits hormone production in an attempt to slow the growth of tumors or cancer cells. It worked for a few months, but after a year Richard’s tumors were growing again.
In September 2017, Dr. Wissel took Richard’s case to the neuroendocrine tumor board, a group of experts in NETs who meet to discuss each patient’s treatment and together come up with the best plan. The tumor board recommended surgery to remove about half of Richard’s pancreas, spleen, gallbladder and partial liver resection. They also did radiofrequency ablation (RFA) treatment on tumors in his liver that they could not remove.
"It was a very big surgery. Even though it wasn’t going to cure me, they said it should help me feel better, slow the spread of the disease and just buy me more time," says Richard. He adds that he had complications after surgery, but the procedure did improve his quality of life and alleviated the stomach pain. Most importantly, surgery removed all the tumors in his pancreas — today there is still no tumor regrowth in that area.
Unfortunately, the cancer continued to spread in Richard’s liver. But his doctors were ready with more treatment options. In September of 2018, they put him on peptide receptor radionuclide therapy (PRRT). This targeted therapy injects radioactive isotopes into the body to attack and destroy tumor cells.
"It made me sick, but it was a treatment option that I was happy to get. Penn was one of the few hospitals that actually offered it at the time," says Richard. His tumor growth was stable for several months, until January 2020 when scans showed progression again.
The next line of attack was TACE — the same treatment his original doctors recommended first. Richard joined a clinical trial, and in February 2020 got chemoembolization on the right lobe of his liver where tumor growth was the most active.
Another Bump in the Road
Soon after his TACE, a completely unrelated complication came up — Richard was diagnosed with prostate cancer. "I am one of the unlucky people that have gotten two cancers," he says. His doctors decided to pause Richard’s NET treatment to address the prostate cancer.
He had a radical prostatectomy (prostate removal) in August 2020, which was partially delayed because of COVID-19. Meanwhile, his NET team was keeping an eye on the other disease progression. During this time he also started seeing a new Penn oncologist, Jennifer Eads, MD. "You talk about baptism by fire — not only is she coming up to speed on my neuroendocrine cancer, but here I am with prostate cancer as well," says Richard.
More Treatments and More Hope
In the months after successful prostate cancer surgery, Richard’s scans showed more liver progression. And his physical health was at an all-time low. "I don’t want to say I was bedridden, but I spent a lot more time sitting than standing. I just felt bad and really couldn’t do a lot," he remembers.
His oncology team put him on an oral chemotherapy called CAPTEM, with transarterial radiation embolization (TARE) at the same time. TARE inserts tiny radioactive beads into the blood vessels supplying a tumor to treat the tumor but not the healthy tissue around it.
Simultaneously, Penn interventional radiologist, Michael Soulen, MD, was leading an active clinical trial to study the benefits of these two therapies together. Richard didn’t qualify for the trial, but his doctors put him on the dual regimen anyway.
Richard says the treatment was hard, but he completed it in January 2021. By April, he was feeling more like himself. His oncologists decided to stop CAPTEM and monitor his tumors. Stopping the treatment early would leave it open as an option again in the future if necessary.
"I’ve burned through a lot of the treatments that are available for neuroendocrine cancer. There’s not a whole lot left out there," says Richard. He feels healthier now, and his tumors are stable. He likes knowing there are still options open to him if they start to grow again.
Richard also knows that his team is invested in his outcomes. "You know that Penn is a world-class neuroendocrine tumor center. The doctors are obviously very busy and have a lot of patients. But the time they spend individually with me is incredible, really."
More than anything, Richard and Peggy still have perhaps the one thing that matters most in a hard fight with cancer. "The doctors at Penn are extremely positive, always talking about what they can do in the future," he said. "They give you hope."