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Saving a Life Against the Odds

Chordoma: A One in a Million Cancer

Rimas V. Lukas, MD Neuro-Oncology

Roger Stupp, MD Neuro-Oncology

Craig M. Horbinski, MD Neuropathology

Borislav A. Alexiev, MD Pathology

Jean-Paul Wolinsky, MD Spine Surgery

Sabine B. Sobek, MD Hospital Medicine

Gregory A. Dumanian, MD Plastic Surgery

Imagine a dandelion.

When you blow on the seeds, they fly away, self-plant and begin to grow where they land. Likewise, a chordoma is a cancerous tumor that can “seed” its cells to surrounding tissues. If a tumor is ruptured in surgery, tumor cells will spill into surrounding areas, planting new seeds that could also grow into new tumors.

If a chordoma is removed with even a single cell left behind, the chance of recurrence is exceptionally high. Ultimately, this means if you can remove the tumor in one piece, without spilling tumor cells, the less likely the cancer will come back, and the best chance you have for long-term survival.
–Jean-Paul Wolinsky, MD

But specialists are rare.

This was the picture painted for Jeff when his initial care provider suspected a chordoma might be at the root of escalating nerve pain in Jeff’s back, along with other physical irregularities. As rare as chordomas are, neurosurgeons with the extensive training and experience required to remove them are even more uncommon. Luckily for Jeff, he was referred for treatment at Northwestern Memorial Hospital, one of the few facilities in the world with a surgeon capable of performing the delicate 14-hour operation necessary to completely remove his chordoma.
spine with chordoma
Patient video background Image of patient

MORE THAN A RACE, THIS WAS A MARATHON.

northwestern medicine chordoma team members standing together northwestern medicine chordoma team members standing together northwestern medicine chordoma team members standing together northwestern medicine chordoma team members standing together

A ONE IN A MILLION TEAM

When internationally renowned neurosurgeon Jean-Paul Wolinsky, MD, joined the Lou and Jean Malnati Brain Tumor Institute of the Robert H. Lurie Comprehensive Cancer Center of Northwestern University at Northwestern Memorial Hospital in 2017, it was precisely for a procedure like this. As chordomas only occur in one out of a million people, finding a care team with experience removing one can be difficult. But Dr. Wolinsky would come to find that his new colleagues not only suited his objective; they exceeded his expectations entirely.

The depth of clinical expertise at Northwestern Memorial Hospital is hard to match. Nearly 30 surgeons, along with supporting staff, consulted on Jeff’s chordoma removal.
–Jean-Paul Wolinsky, MD
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MEETING OF THE MINDS

Rimas V. Lukas, MD Neuro-Oncology
Roger Stupp, MD Neuro-Oncology

As members of the Northwestern Medicine Neuro-Oncology tumor board, Rimas Lukas, MD, and Roger Stupp, MD, regularly consult with their colleagues on cases like Jeff’s. The board — consisting of internationally renowned neurological oncologists, pathologists, surgeons, radiologists and other hospital staff — meets weekly to review cases and scans to form the best diagnosis and treatment plans for patients who need complex care.

We believe that the tumor board’s review of complex cases helps Northwestern Medicine elevate patient care to the next level.
–Rimas Lukas, MD

Often the scene of inquiry and debate, insight shared at these gatherings strengthens treatment throughout the Neurology and Neurosurgery units. From providing surgical consultation to advising on radiation therapy, even when Dr. Lukas and Dr. Stupp never directly treat a patient like Jeff, they help influence and navigate that patient’s care plan.

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SAVING A SUPERHIGHWAY

Jean-Paul Wolinsky, MD Spine Surgery

Chordomas are slow-growing tumors, which means it can take months or years for them to become identifiable. But what Dr. Wolinsky saw in Jeff’s scans convinced him that it was a chordoma beyond a shadow of a doubt. Wrapped around three vertebrae was a soft-tissue sarcoma — a cancerous tumor that forms near connective tissue — threatening to metastasize, or spread to other parts of the body. In certain scenarios, specialists will monitor a growth to see whether it continues growing or risks encroaching on surrounding tissue, but Jeff’s had already spread across a dangerous amount of his spine. Slowly, over the course of a 14-hour surgery, Dr. Wolinsky and his team went to work.

Your best shot is the first shot. Getting it out in one piece gives a patient the best chance for disease-free survival or a cure.
–Jean-Paul Wolinsky, MD

The procedure, better known as a “thoracic spondylectomy,” began with the removal of the three vertebrae (nearly 6 inches) from Jeff’s spine. In place of the vertebrae, Dr. Wolinksy inserted metal rods and a cage to reconstruct the spine, as well as to prevent any neurological damage. For periods of time during surgery, Jeff’s spinal cord was totally exposed, basically floating in his body, as his chordoma was excised. Finally, as he had done more than 100 times before, Dr. Wolinsky confirmed that Jeff’s mass had been removed.

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CLOSING THE BOOK

Gregory A. Dumanian, MD Plastic Surgery

Still, Jeff’s journey was far from over. Due to the size and location of Jeff’s surgical incision, Plastic Surgeon Gregory Dumanian, MD, was brought on to the team to close up the wound. Observing Dr. Wolinsky’s work in the OR allowed Dr. Dumanian to plan exactly which tissue would be grafted to ensure coverage and future mobility.

The process of closing the wound takes many things into account: what’s missing, how much tension is acceptable at the wound site and so on. We have to find our closure through parallel problem-solving.
–Gregory Dumanian, MD

In what’s called a “prophylactic closure,” Dr. Dumanian stitched the wound closed using muscle tissue from the back and shoulder. Finally out of surgery, Jeff began a slow, careful period of physical therapy, closely supervised by Dr. Dumanian to ensure proper healing. Meanwhile, Sabine Sobek, MD, and the perioperative team monitored Jeff’s post-surgery care and pain management. This multidisciplinary team approach led to a successful outcome as Jeff is now back at work and living a physically active lifestyle, despite a large section of his spine having been replaced with a metal cage.

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FINDING MOLECULAR CLUES

Craig M. Horbinski, MD Neuropathology
Borislav A. Alexiev, MD Pathology

The only way to definitively diagnose a chordoma is to view its cells under a microscope. However, due to the “flowering” nature of chordomas, taking a biopsy of Jeff’s tumor before operation would have been incredibly risky due to the threat of spilling rogue cells. Thus, after surgery, once Jeff’s mass was removed, it was entrusted to Soft Tissue Pathologist Borislav Alexiev, MD, and Surgical Neuropathologist Craig Horbinski, MD, director of the Northwestern University Nervous System Tumor Bank, for complete analysis.

Pathology plays ‘umpire’ in the world of tumors. It’s not until we view a tumor under the microscope, and perform our genetic testing, that we can make our final call.
–Craig Horbinski, MD

Finally able to view Jeff’s cells up close, Dr. Horbinski and Dr. Alexiev found a significant expression of brachyury, a protein highly indicative of a chordoma. Since 2014, the Northwestern University Nervous System Tumor Bank has collected more than 2,300 biospecimens from donors for medical research. And now, after finally being removed, Jeff’s chordoma is powering research that might one day help researchers tackle cancer at its roots.

digital image of a chordoma and it's treatment
The two words that would characterize our team, specifically in the brain tumor and spinal tumor setting, would be ‘respect’ and ‘trust.’ At Northwestern Medicine, there is a freedom to be honest when something is difficult, because everybody recognizes that medicine is challenging.
–Craig Horbinski, MD

What makes us better, makes you better.®

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LEADING BY EXAMPLE

At any given time, fewer than one in 100,000 people worldwide may be living with a chordoma. But throughout the Northwestern Medicine system, preparedness to treat any patient with any illness is core to the mission.

I think that is what’s invigorating about something like the tumor board, having the opportunity to spar with top-tier colleagues to improve or pioneer the best possible outcomes for our patients. You’ve gotta bring your A game.
–Rimas Lukas, MD

With his colleagues, Dr. Wolinsky continues to explore new opportunities for chordoma treatment, from utilizing 3-D printed bones as a potential substitute for titanium reconstruction to continued advances in proton therapy and the use of novel drug therapies. Though chordomas may be rare in the general population, they are not rare to the Northwestern Medicine specialists paving the way for better outcomes for patients.

Northwestern Medicine physicians looking at an X-ray. Image has a white overlay on top.