Her Sister Died of a Brain Tumor. Now She Was Having Similar Symptoms.
I knocked and entered her room. Our patient had a few more questions about her surgery.
I introduced myself.
“This isn’t the same tall guy as before,” I recalled her joking.
“They only hire tall people,” I said.
Wearing masks in every hallway and patient room, though necessary in the time of Covid-19, had stolen the familiarity of a smile.
She was in her late 50s, wearing glasses and a light blue hospital gown, sitting up in bed. She had a full head of salt and pepper hair that was more salt than pepper really. Sprawled across her beige blankets were legal pads on which she had been taking notes.
On her right was a gingham-lined picnic basket brimming with treats, Coca-Cola and Otterbein’s Cookies, a local favorite, gifts from her co-workers.
A few weeks earlier, her life had been routine. As an information technology specialist, she had been working from home,. At some point she realized she had been experiencing weakness in her right leg, and then in her right arm.
“I noticed it late, I think, because I was sitting at my desk all the time,” she said.
Her handwriting had worsened precipitously over the previous few days. “I can’t write anymore,” she said, “and when I was lifting weights my arm felt heavy and weak.”
She had gone to the emergency room, where doctors performed a CT scan of her head. On the left side, at the top of her brain, they found a “hypodensity,” a dark area that would need further work-up. A subsequent M.R.I. scan of her brain provided more clarity.
A cystic mass was invading her left paracentral lobule, part of her primary motor cortex, a one-centimeter ribbon of brain tissue packed with the millions of neurons that allow us to voluntarily control our muscles.
I asked her if she had more questions about her surgery, which was scheduled for the next day. My attending had talked previously with her about the finding on her scan and options going forward.
“I’m just wondering what the mass is,” she said. “Hopefully you won’t find anything,” she followed up quickly.
“A scan is a representation,” I said. “We can only know for sure once we look at a piece of it under a microscope, after the surgery.”
Our concern was a tumor, but the mass could also be an infection, or an inflammatory process, or another disease, I explained. As we talked more, I learned more about the source of her anxiety.
Her sister had died of a glioblastoma a few years earlier, she told me. Glioblastoma is the most common tumor that arises from brain tissue, and one of the most lethal of human cancers. Three people in every 100,000 are diagnosed with this condition every year.
She recounted the story of her sister’s diagnosis and treatment, and the misery she had gone through. Her sister, too, had a mass on the left side of her brain, for which she had elected surgery. The surgery and mass had left her weak and unable to speak. After the diagnosis of glioblastoma was confirmed, she had undergone chemotherapy and radiation therapy, the standard of care, which can extend survival a few months. She died a year later, and had never regained her old self.
“Me and my sister have everything in common,” she said. “When I heard about this mass, I said, really, we have to have that in common too?”
I smiled at her efforts to add some humor to the situation.
“I read that glioblastoma is extremely rare, so it’s unlikely I have the same thing, right?” she asked.
I explained it was rare, but possible.
“My sister wasn’t the same after surgery,” she said. “Don’t take so much out that it causes problems.”
We talked a while longer before I left for the evening.
The next morning, the chief resident and I greeted her in her room.
“I’m ready,” she said.
Her nurses brought her down to the operating room, where the surgical nurses and technicians began their rhythms, making her comfortable on the operating table, moving equipment.
The anesthesiologist skillfully intubated her. He ferried her mind to the hinterlands.
We started by cutting a narrow furrow through her hair, as she had requested, preserving as much hair as possible. Cutting the hair before making an incision is a small part of brain surgery, but it remains our most visible maneuver. If she had what her sister had and we’d cut it all, her hair might never fully grow back in time. She would forever be marked by her disease. Cutting less mattered to her.
My attending, the chief resident and I cleaned her hair and scalp, and then, using the surgical drapes, created a window where we would work.
I made an incision down to her skull with a scalpel, and we instinctively stemmed any bleeding. After folding her scalp back and exposing her skull, I made several burr holes with a drill, then connected these using a different kind of drill to complete our craniotomy, removing a disc of skull. My chief resident and I then cut and drew back the dura, the pearly lining that protects the brain. We were now staring directly at her brain.
Using surface electrodes, and tiny jolt of electricity, we determined the boundaries of her motor cortex, the no-go zone. Compromising any part of this ribbon would result in paralysis.
My attending and chief resident started dissecting between both brain hemispheres. They met the mass from its flank. It was aberrant, alien.
We sent the first piece of it to the pathologist for analysis.
They methodically resected the mass, working from within the abnormal tissue itself; better to damage the mass than to harm healthy brain tissue. They peeled it away from her brain, bit by bit. It was deliberate work.
The phone rang, interrupting the sound of buzzing electrocautery forceps and whirring suction tips. It was the pathologist.
“The specimen is consistent with glioblastoma,” her voice rang on speakerphone, to a change of tenor in the room.
Like her sister, I thought to myself.
Two years earlier, I had seen Damien Chazelle’s “First Man,” a biopic of Neil Armstrong. There was a moment in the film when the astronaut stood at the rim of a baby crater on the moon. He had spent 10 minutes at that crater, but what he did there remains a mystery. The filmmakers had suggested he left a bracelet there, a bracelet that had belonged to his daughter Karen, who had died seven years earlier, in 1962, at age 2.
Karen had succumbed to what is believed to have been diffuse intrinsic pontine glioma, a rare childhood cancer of the brainstem that shares some similarities to glioblastoma. His daughter had undergone radiation therapy, which reduces symptoms from the tumor, but only for a short while. It remains the only available treatment for this devastating disease, which today, as then, is almost universally fatal.
Humanity has planted flags on the moon, yet a moonshot for brain cancer has yet to be realized.
Diagnosis known, we gradually stopped removing more tumor. The more tumor you remove, the longer the average survival, meager though it may be. But the quest for surgical perfection sometimes comes with a cost. In the brain, where critical human functions are packed into mere millimeters of tissue, removing more tumor and possibly damaging healthy tissue risks the loss of strength, speech, vision, memory and more. In glioblastoma, tumor cells that have traveled centimeters away from the bulk of the tumor, far out of reach of any forceps, almost guarantee the cancer’s recurrence. Surgical perfection is imperfect. She wanted to preserve her strength.
We sutured the dura closed, and then plated her bone back on. With care, we closed the layers of her skin. In a short while, she was extubated, and we brought her up to our neurological intensive care unit to recover.
“I have seven years on my sister, and a lot of young people are dying these days, so I’m trying to be pragmatic,” she had said to me the day before. Bargaining.
Forty years ago, the median survival time for glioblastoma was four-and-a-half months. Since then, researchers have characterized the genetics of glioblastoma and studied various vaccines, chemotherapies, immunotherapies, cell therapies, new imaging modalities, targeted radiation therapies and innovative forms of drug delivery to treat the disease. Many steps.
The median survival time today is about 15 months. Only a small percentage of patients survive more than five years.
Defeatism is a common feeling among neurosurgeons, but you maintain resolve, for your patients, and for yourself. The next morning, our patient was in good spirits, recovering well, with good strength. We delicately shared the diagnosis with her.
“Just my luck,” she said with a smile. She seemed to be expecting it.
Some cancers in siblings can be explained by genetics. But that’s not the case for glioblastoma. As for her sister, and many others, it really was just bad luck.
Our surgical team, along with our colleagues in neuro-oncology and radiation oncology, recommended the standard treatment of chemotherapy and radiation therapy. But she declined treatment multiple times and chose palliative care.
“I don’t want to drag it out,” she said.
She died peacefully four months later, surrounded by her family, listening to “The Sound of Music,” her favorite musical.
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