A Charley Horse Led to Pancreatic Neuroendocrine Tumor Discovery

Mark Head

• CT scan for massive blood clot in my leg finds a tumor on my pancreas
• Biopsy reveals neuroendocrine tumor rather than adenocarcinoma
• Chemotherapy clinical trial followed by Whipple procedure
• Maintenance treatment

In December of 2014 I was shoveling snow from a 15-inch winter burst, which is pretty typical for Rochester, New York, during the winter months.

About twenty minutes into this rueful chore, the unthinkable happened. For approximately 20 seconds I could not breathe. My lungs would not function. I wrongly surmised that I was experiencing a heart attack. But as abruptly as it started, it ended and I was able to breathe normally once again. The only noticeable side effect was acute fatigue.

A subsequent trip to my primary care physician’s office at my (obviously more in touch with reality) wife’s urging led to a cannonball run to the emergency room, which revealed a massive blood clot—a deep vein thrombosis (DVT)—in my left leg, from my left ankle all the way up to my groin. I had naively passed off the knot in my calf as a “charley horse,” although it had been present for approximately three weeks. The ER staff was most concerned with the prospect of another DVT clot breaking off and causing pulmonary and heart problems. I was placed on a blood thinner and an interventional radiologist was dispatched to insert a filter into my vena cava in order to “catch” any clot(s) that broke off from the main one.

From a Big Problem to a Giant Problem

In the search to discover the cause of the clot, I had a CT scan. A scant two hours after the CT scan was performed I got a phone call from my doctor’s office requesting that I come to the office as soon as possible to discuss my results. There was that foreboding caveat attached “bring your wife with you,” which made my heart race. That sentence was a harbinger that things were about to change forever.

The scan revealed a 9 cm malignant mass on the head of my pancreas. Initially the tumor was identified as an aggressive adenocarcinoma and I was told categorically that I likely had three to six months to live and to “get my affairs in order.” I was only 59 at the time but I put in my retirement papers immediately, in order to protect my pension in the event that I died suddenly.

A biopsy was scheduled for two days later. At this meeting Dr. Ari Chodos, an astute gastroenterologist deduced that the size of the mass and its location at the head of the pancreas in all likelihood should have made me much more symptomatic: jaundice, bile duct blockage, bowel obstructions are all frequently present with an adenocarcinoma this large. Based on the atypical presentation of my tumor, this doctor was hopeful that the first diagnosis was erroneous.

The biopsy (done through an esophageal ultrasound) performed revealed that I had a much rarer tumor known as an undifferentiated neuroendocrine pancreatic mass. This was similar to Steve Jobs’ tumor, from which he eventually died.

This was one of those good news/bad news discoveries. Yes, the mass was malignant and it was inoperable due to the tumor’s encroachment on the mesenteric vein and artery. No, it was not the aggressive type first surmised. It had likely been present for a number of years, and had recently become symptomatic, by causing DVT clots in the lungs. If not for that very scary blood clot incident the tumor might have gone undetected for a longer time.

Now I had to decide on whether to engage in treatment and if so, which course. The neuroendocrine tumor is considered to be a zebra among horses—it is extremely rare and because of that chemotherapy options are severely limited.

Making Treatment Choices

My next move was to huddle with my wife and children (young adults ages 22, 24, and 25) in Florida to discuss these life-changing events. While we were there, Dr. Chodos phoned and confirmed that the tumor was indeed a neuroendocrine mass. He promptly referred me to the Wilmot Cancer Institute, which had a clinical trial of capecitabine and Temodar (CapTem) chemotherapy. I was told that if this didn’t work my options were limited. I also started monthly Sandostatin (synthetic hormone) injections to try and keep the tumor from growing.

My wife researched my tumor and suggested that we get in touch with Pancreatic Cancer Action Network (PanCAN). I contacted them and talked to people in their Know Your Tumor program. They confirmed that the chemo cocktail used in the trial was my best hope for treatment.

Luckily for me, after nine months on the CapTem regimen of two weeks on and two weeks off, my tumor had shrunk 40 percent. A regimen of stereotactic body radiation therapy (SBRT) was added. I was once again reviewed by the Wilmot Cancer Institute’s tumor board, hoping to qualify for a Whipple procedure. The board declined to resect the entire tumor due to encroachment on the mesenteric vein and artery. Frustrated and still determined to undergo a Whipple procedure, I sought a second opinion from Johns Hopkins University Cancer Center. Two weeks after reviewing my records, Dr. Marty Makary (Chief of Islet Transplant Surgery) gave us the good news—he agreed to do the surgery.

Dr. Makary believed that this mass had to be removed and even if it couldn’t be entirely resected, he was confident that he could remove 95 percent of it. I underwent Whipple Surgery at Johns Hopkins on March 24, 2016. The surgery lasted eight and a half hours and Dr. Makary removed 95 percent of the tumor. Eighty staples later I was on opioid pain medication, although after eight days, my pain level was well under control and I switched to extra strength Tylenol only. I suffered no clinical complications with the exception of a low fever. Fourteen days later I was discharged from Johns Hopkins minus my gall bladder, my duodenum, 40 percent of my pancreas, part of my small intestine, and part of my stomach.

My oncologist is Dr. Mohamed Tejani, at Wilmot (Dr. Tejani is now at AdventHealth in Orlando, Florida). After my Whipple, my blood work was monitored monthly and for the first eighteen months I had CT scans every three months to monitor possible recurrence or progression in the surgical bed. In addition to these safety checks I still receive monthly Sandostatin injections in the hope of keeping the remaining tumor at bay.

Life in Stage IV

My status is considered to be stage IV—treatable but not curable. Besides the remaining 5 percent of the tumor, I have three spots on my liver although they have not progressed. I became diabetic as a result of the surgery and must take metformin and insulin. I also take Creon (enzyme replacement) with every meal, as well as a blood thinner. Plus my monthly Sandostatin injection. My new normal is very active physically and a slightly modified diet.

What are my takeaways?

• Always seek a second opinion from a large volume cancer center—your life hangs in the balance.
• If you are eligible for a surgery like the Whipple, have it performed at a large volume center like Johns Hopkins, Memorial Sloan Kettering, Dana Farber, or MD Anderson. Why? The complication rate, and mortality rate are lower. The surgeons are far more practiced in doing the procedure.
• Limit the opioid medication for pain management. Opioid effectiveness diminishes over time.
• Don’t view cancer, even pancreatic cancer, as a death sentence. Clinical trials, immunotherapy, and new techniques in palliative care make living with cancer more manageable.
• Utilize support systems, meditation, Facebook cancer support groups specific to your kind of cancer. They are wonderful in comparing notes and learning from others further down the road. They can provide spiritual and emotional support. Contact PanCAN; they are a very helpful resource.
• Talk openly with family about your end of life preparations. Stay connected to living your life.
• Balance your role as cancer patient with that of your role as a life-long learner and lover of life.

And most of all . . . Never Ever Give Up!