Here she is, the unicorn.
Only Lynn Zimmer didn’t quite know it yet.
In the early months of 2019, Lynn was having a nagging pain that just wouldn’t go away. She chalked it up to issues with her gallbladder but decided it was still worth checking out. Lynn went to see her primary care provider at Mountain View Health Practice in her hometown of Grants Pass, Oregon.
An ultrasound revealed a tumor, located in her common bile duct – a tube that carries bile from the liver and gallbladder, through the pancreas, and into the small intestine. The tumor was causing a backup in the gallbladder – the culprit of this newfound pain.
Immediately, Lynn’s care team was on the phone putting in a referral to the OHSU Knight Cancer Institute.
Lynn recalls her doctor saying they couldn’t think of any place else to refer her.
On the receiving end of that phone call was Dr. Skye Mayo, a surgical oncologist specializing in Hepato-Pancreato-Biliary Oncology (HPB Oncology) – meaning cancers of the liver, pancreas, and bile duct. Dr. Mayo remembers that day very clearly.
“I was in the operating room, and I had just finished a case when I got a call from the OHSU operator about a doctor that wanted to speak with me, who just saw this patient in the clinic and needed some advice,” recalled Mayo. “I remember pulling up the scan and seeing this, and immediately two things popped up when I saw this mass: either this patient has a history of melanoma, or this is a neuroendocrine tumor that’s spread to her bile duct.”
Dr. Mayo reviewed Lynn’s imaging and quickly briefed his team.
“I knew it looked like something that was going to be atypical,” said Mayo.
An already atypical diagnosis became more complex given Lynn’s history with a previous gastric bypass surgery approximately 20 years ago, which would make the route to scope the bile duct for biopsy more “circuitous” and “longer” according to Dr. Mayo.
What was Dr. Mayo’s approach?
Name It. Stage it. Treat it.
In order to put a name to this atypical mass in Lynn’s bile duct, Dr. Mayo and team arranged for a biopsy on March 13, 2019. The biopsy showed it was a neuroendocrine tumor. Neuroendocrine tumors start in cells that have aspects of nerve cells (neuro) and hormone-producing cells (endocrine). These cells release hormones into the bloodstream in response to signals from the nervous system. A tumor forms when these cells begin to rapidly divide and grow out of control. Neuroendocrine tumors can arise from cells found throughout the body ranging from the intestines to the skin.
“It was a low grade and well-differentiated, so the good news was that it was a pretty low-risk neuroendocrine tumor,” added Mayo. “It wasn’t causing any additional symptoms, and it wasn’t blocking off her bile duct. Because of that, I had her get a specific type of PET-CT scan to look for the receptors that are expressed by some neuroendocrine tumors called a Dotatate PET-CT, which is a special scan we do for patients that have neuroendocrine tumors here at OHSU.”
The Dotatate PET-CT scan illuminated the biopsied tumor in Lynn’s bile duct confirming that it expressed the receptors. According to Dr. Mayo, the brightness of the scan was consistent on the imaging with a neuroendocrine tumor, but most importantly, nowhere else in her body showed illumination under the scan – meaning the tumor had not yet spread.
Having a neuroendocrine tumor arising, or starting, from the bile duct is “extremely rare” according to Dr. Mayo. The Dotatate PET-CT scan was only the first measure of confirmation, however. Given the circumstances, it was critical to be as certain as possible. Knowing this, Dr. Mayo honed in for final confirmation.
“The challenge is always to make sure that we have looked thoroughly everywhere else, which I did in the operating room, and with imaging before the operation, to make sure that there wasn’t a very small originating site somewhere else, and then it spread to the bile duct,” added Mayo.
“We also performed a bronchoscopy in the operating room, where we take a camera and look down her airways, to also look for a tumor because one of the common sites can be arising in the airways. We didn’t see any of that.”
No stone was left unturned. The bile duct was the source of this tumor; it was the epicenter.
The plan now was to surgically remove the tumor.
Now it was time to bring in Dr. Robert Martindale, a general surgeon at the OHSU Knight Cancer Institute who specializes in digestive health. Dr. Martindale has expertise in nutrition and helping patients that have had different types of gastric bypass and weight loss operations.
“He’s someone who has pretty much seen everything over the course of his career. I needed his experience in making sure that if I was going to resect her bile duct containing the tumor, in order to remove the tumor, that I was able to reconstruct her intestines and bile ducts given the unique way Lynn’s anatomy had been altered already because of her prior bypass operations,” says Mayo.
Despite the unusual diagnosis and complexities of the situation, Lynn was comfortable and at ease with her care team at OHSU, saying, “between the two of them, I got the best and honestly felt like I was in the best hands.”
There was a particularly strong bond between Lynn and Dr. Martindale that still remains to this day.
“We just had this connection, this understanding, that we’re in this together,” Lynn says. “And I kept telling Dr. Martindale throughout this journey that ‘You’re not going anywhere; you’re not retiring until you finish this up here with me.’ What a hero, what a hero.”
Mayo says he is grateful for Martindale’s expertise.
“That’s one of the benefits of being at a place like OHSU, and being able to have incredible colleagues like Dr. Martindale who are willing to help out.,” he says.
On April 2, 2019, Lynn underwent an operation to remove the tumor from her bile duct. The surgery took over eight hours to complete and was successful, but not without some unique challenges.
“I have been told that I’m uniquely rewired inside,” Lynn says today.
After the successful surgery to remove her neuroendocrine tumor, the road to recovery began. Lynn knew she was in for a challenge: post-surgery recovery was long and arduous, which she described as a “fog.” Hurdles included not being able to keep her nutrition up and getting her mind and body on the same page.
“I had it in my head that I needed to go forward, but there was this feeling of, my mind and my body were just not cooperating. And I say this because my diagnosis came exactly to the date one year after my soulmate, my husband of nearly 50 years passed away,” Lynn recalls.
Lynn says it was her faith in God along with the love and nurturing of her family and physicians that helped keep her pushing forward. It wasn’t easy, she admitted.
“My recovery couldn’t have fallen into more perfect timing,” Lynn says. That summer sun is healing for me and I am looking forward to getting outside, and digging in the dirt. I live on eight acres of beautiful forestry here in Oregon and there’s something so peaceful about watching the deer.”
The weather isn’t all that Lynn has going for her. Lynn recently watched her granddaughter graduate from college virtually on YouTube.
“I’m all fired up for life,” Lynn exclaimed. “I wish I could have been there (in person) for the graduation, but I’m still getting back up to speed and healing. But I have so much to live for.”
To date, Lynn has had no evidence of cancer recurrence and is doing just “incredible” right now, according to Dr. Mayo who continues to monitor and visit Lynn throughout the recovery process.
THE “UNICORN OF ZEBRAS”
In the medical field, the animal representative of rare conditions is often referred to as a “zebra.” Patient support groups and advocacy groups for patients with neuroendocrine tumors have adopted the zebra as their mascot.
Lynn’s neuroendocrine tumor would characterize her as a zebra, but her case was even more rare than that. According to Mayo, it was almost unheard of.
“That is why I referred to Lynn as a unicorn,” says Mayo. “She’s the unicorn of the zebras. A rare, seemingly mythical thing that could happen for her that some people didn’t believe exists. But we showed with our in-depth review of her pathology here at OHSU which we published that she has a true neuroendocrine tumor arising from her bile duct.”
There’s a special bond that forms between patient and provider during a difficult diagnosis. A bond that grows with trust between one another. Trust offers confidence in one another as well as an understanding of a shared common goal.
“Lynn is such a special, wonderful, and a trusting person,” Dr. Mayo says of his patient. “As a cancer surgeon and an oncologist, it’s a gift when you get to work with someone you know trusts you.”
There was no textbook for what Lynn has. The unknown can become overwhelming when there are no step-by-step instructions to follow, because the occurrence is so rare.
“She just makes my day,” Mayo says. “Whenever I see her in clinic, she’s usually wearing shirts with a zebra along with a unicorn headband. She has a great personality – she’s very optimistic, upbeat, and just an absolute pleasure to take care of.”
“I am feeling so awesome and feel so blessed that I believe we have closed this chapter. I am a very fortunate individual,” said Lynn. “Here I am, the unicorn.”