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How we did it: The World's First En Bloc Resection of a Rare Cervical Chordoma Tumor

A car crash saved Noah Pernikoff’s life.

When the 27-year-old New Yorker developed a nagging neck pain after the accident, an x-ray revealed a concerning lesion in his neck on his cervical spine. Completely unrelated to the accident, the lesion turned out to be a chordoma. His physicians had caught it early, and thankfully so—many chordomas can be life-threatening in their later stages.

Chordoma Diagnosis

Skull-base chordomas are rare, occurring in roughly one in one million patients. Cervical chordomas comprise about one-third of chordoma cases. While skull-base chordomas are technically benign, they can still aggressively invade surrounding areas. Due to their dense and fibrous nature, an untreated chordoma can compress the brainstem and have deadly consequences.

“The physician said if I hadn’t discovered it through the car accident, it probably would have kept growing until it came to a point on my spinal cord where it caused paralysis or death,” Pernikoff said. “I feel very lucky in that regard.”

Chordoma Treatment

Chordomas carry a high risk of recurrence if not fully resected. The location of Pernikoff’s chordoma—on the C2 vertebrae—meant surgery would be incredibly difficult. Pernikoff’s neurosurgeon recommended radiation with proton therapy and referred him to Penn Medicine. Penn offers exclusive access to proton therapy in the region.

Without Any Surgical Options, Penn Had to Invent One

As a national leader for complex spinal surgeries, Penn is one of the few facilities nationwide that has the expertise and technology to treat chordomas.

In Pernikoff’s case, our multidisciplinary team of neurologists and otolaryngologists understood that radiation wouldn’t be enough to address his chordoma. Knowing that surgery was a must, our team pooled their knowledge and skill to create a first-of-its-kind surgical procedure that could fully resect the chordoma while offering improved healing and recovery times.

A Collaborative Clinical Team 

The multidisciplinary team that performed the surgery was led by Neil Malhotra, MD, an assistant professor of Neurosurgery and Orthopaedic Surgery and the vice chair of operations in the Department of Neurosurgery. To create this new kind of surgery, Dr. Malhotra formed an innovative partnership with Bert W. O'Malley Jr., MD, a professor and chairman of the Department of Otorhinolaryngology: Head and Neck Surgery.

Dr. O'Malley recommended using a trans-oral robotic surgery (TORS) for part of the procedure.

A Surgery with Trans-Oral Robotic (TORS)

Invented at Penn, TORS is the world's first group of minimally invasive robotic surgery techniques to remove benign and malignant tumors of the mouth and throat.

Before TORS, chordoma patients had to endure major invasive surgery. This includes splitting the jaw bone, making major cuts in the chin, neck, throat, tongue and palate, and potentially destroying speech, the ability the swallow, and cosmetic appearance. By simply working through the mouth, TORS sidesteps all of these major issues while helping neurosurgeons visualize the approach and closure during their resection. 

En Bloc Resection of a Cervical Chordoma 

The procedure developed at Penn was called En Bloc Spondylectomy and TransOral Robotic Surgery. While proton therapy is an excellent second treatment option, en bloc resection offers chordoma patients the best chance of a cure.

“En bloc resection for cervical chordoma is among the very few procedures in surgical practice with no margin for error," said Dr. Malhotra. "But there was no question in my mind, or Dr. O'Malley's, that we could perform this surgery. Working within the confines of perfection is what training, deliberative planning, and innovation are about."

The En Bloc Spondylectomy and TransOral Robotic Surgery procedure consisted of three main stages:

  • Stage I In the first stage, Dr. Malhotra and a team of neurosurgeons entered the back of Pernikoff’s neck and separated the tumor from the spinal bone. There were two critical goals during this stage: first, surgeons had to make ultrasonic bone cuts around the tumor without actually touching it. Second, they had to avoid injuring the spinal cord that lay between the surgeon and the tumor/spinal column. 
  • Stage II In the second stage, Dr. O’Malley and a team of head and neck surgeons used TORS to clear a path so the tumor could be completely visualized, enabling Dr. Malhotra to remove the tumor, along with a small part of the spinal column, en bloc. A second TORS procedure was performed to close that pathway to the tumor.
  • Stage III Finally, the team reconstructed Pernikoff’s spinal column. Because they had removed an important bone in his neck, they used some of Pernikoff’s own bone from his hip and rods to finalize stabilization of the newly built portion of his spine.

Success: The World's First En Bloc Spondylectomy and TransOral Robotic Surgery

The surgery was successful and free of complications. Pernikoff was sent home after six days in the hospital. He has since returned to work, and to date, there is no evidence of tumor recurrence or complications as a consequence of his surgery.

Only Penn’s collective expertise, innovative spirit, and culture of collaboration could have produced an entirely new procedure to overcome the challenges presented by a single patient.

When you refer a patient to Penn, our collaborative, team-based approach ensures that your patient will get to the right provider, whether it’s a noted expert in their field or a multidisciplinary team tackling complex or unique cases.

Additional Chordoma Resources from Penn Medicine

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The Penn Physician Blog is a resource for health care professionals featuring Penn Medicine physicians and their research, innovations, programs and events. 

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