Every time Aaron Agler recovered from a sinus infection, it returned. Time after time he visited his doctor for antibiotics, but the infection always came back. “It got to the point where the doctor was like, ‘OK, no more antibiotics.
Something else is going on here,’” Danielle Styer, 31, of Cortland, Ohio, tells TODAY.com. Agler’s various treatment makes it difficult for his voice to carry so Styer, Agler's girlfriend, answers questions for him.
“When he looked in his mouth ...
it looked like the roof of his mouth was heavy, like pushing down.” Eventually Agler, then 27, learned he had nasopharyngeal rhabdomyosarcoma in his sinuses and mouth, a rare soft tissue cancer that normally occurs in children, according to the . “It was a children’s cancer,” Styer says.
“Where it was in Aaron was super rare. It was (located) in prime real estate is what they kept telling us.” On top of having sinus infections that would not subside, Agler, now 34, also “started to snore out of nowhere,” in 2017.
Doctors wondered if he had sleep apnea. Eventually doctors used a scope to examine Agler and found that he had nasopharyngeal rhabdomyosarcoma in February in 2018. Doctors created a treatment plan that included 40 weeks of chemotherapy with six weeks of radiation after the first three months of chemotherapy.
“By fall they said that everything should be fine or should be getting better,” Styer says. “They would have a better idea of what (treatment) should be next.” But when Agler met the 12-week mark to start radiation, scans revealed that the cancer had continued to grow.
Doctors gave Agler a tracheostomy — an opening in the windpipe that assists in breathing — in case radiation to his head and neck impacted his ability to swallow. He finished six weeks of radiation in summer 2018 and began a different chemotherapy regimen. “The tumor started gradually shrinking,” Agler tells TODAY.
com via email. “For almost a year it continued to shrink to the point where they felt comfortable removing my trach.” About a year after diagnosis, Agler returned to work again.
Driving home from work, he yawned while looking at himself in the rearview mirror and was stunned. “You could physically see the tumor,” Styer says. “It was coming down and you could see it in the back of his throat.
” The couple worried. “It was actually really scary because when you think about it unless it’s like a skin cancer ..
. you don’t see a tumor,” Styer says. “It was really unnerving.
” This new tumor grew so rapidly that doctors gave Agler another tracheostomy and a feeding tube. But doctors worried this mass could harm Agler. “They were afraid it was going to cut off his airway,” Styer says.
“It started growing around his carotid artery.” While Agler believed his cancer responded better to radiation, it came with risks. When doctors use radiation on tumors, the tumors can first balloon up before shrinking and doctors worried that could impact his ability to breathe.
What’s more, his mass was nestled in the back of his mouth, close to his spine and brain and radiation could cause damage to important structures. Surgery also seemed dangerous because of the tumor’s grasp on the carotid artery. In summer 2019, they met Dr.
Shlomo Koyfman, a Cleveland Clinic radiation oncologist, who agreed to give Agler a massive dose of radiation in the hopes that it could shrink the tumor for it to become small enough for surgical removal. At the last minute, though, doctors were hesitant about performing surgery. “He literally could die in the surgery because of how dangerous it is,” Styer says.
“The day before (doctors said), ‘Sorry guys, we can’t do it. It’s too dangerous.’” The doctors decided not to go through with the surgery because they didn’t think they could get clean margins.
The couple felt devastated but understood. “We didn’t know how to tell the family because everyone was preparing for all of this,” Styer says. “How am I going to tell his mom that he’s not going to have surgery to try to save his life?” Doctors put him on a new chemotherapy regimen.
At first, it seemed like the cancer was going away. But the couple wasn't sure if the big dose of radiation prior to surgery or the chemotherapy was helping him. “Even the doctors were like, ‘It’s so hard to know which one is doing what or if both are helping each other,’” Styer recalls.
Like clockwork, though, the cancer started growing again after six months of chemotherapy. While they left Ohio for a second opinion, Agler simply received the same treatment at the new hospital and decided to return to the Cleveland Clinic. But this time, he started seeing a pediatric oncologist, even though he was an adult.
“You could tell (the doctor) wasn’t going to give up on anything,” Styer says. “He was bound and determined. He said, ‘This is a challenge for me.
’” The pediatric oncologist offered “more hope” than Agler's previous doctors. “You could just tell he was not going to let Aaron die,” Styer says. “Everything kind of turned and it felt better.
” The new doctor reached back out to Koyfman to see if Agler could have another radiation treatment. Agler thought he had as many radiation sessions as possible and might not be a candidate for another treatment. Still, Koyfman recommended five treatments over two weeks that were so intense it would “most likely create a hole in the back of (his) throat,” Agler says.
That meant he’d have to undergo reconstructive surgery using a tissue from his leg to fix it. This treatment is called stereotactic body radiation therapy (SBRT). “Koyfman gave us a lot of details (about) what could have happened.
It could kill everything around the cancer. It could kill the good (and) the bad. It’s super dangerous,” Styer says.
“It was super precise though. So it didn’t hit the brain. It would just hit that area.
” Knowing his options were limited, Agler agreed to go through with it. “It killed (tissue) all the way to his spine. He had an infection,” Styer says.
“That was the only time Aaron every complained about being in pain. He couldn’t do anything the pain was so excruciating.” Sarcomas are cancers that form in the bones, fat and muscle, and their treatment differs from solid tumor therapies, Koyfman says.
“A typical sarcoma is actually not very sensitive to chemotherapy,” he tells TODAY.com. “Usually, we treat them with surgery and sometimes radiation.
” Agler’s cancer, nasopharyngeal rhabdomyosarcoma, differs because normally it “is exquisitely sensitive to both chemotherapy and radiation. So, we actually rarely cut them out,” Koyfman says. “It’s very aggressive, but also quite sensitive to treatment.
” Most patients receive chemotherapy for a year with five or six weeks of low-dose radiation. But Agler’s tumor didn’t respond to chemotherapy. “It actually didn’t budge,” Koyfman says.
“He will be the first one to tell you that he never felt that chemo worked, which is unusual.” While radiation helped him originally, doctors were wary about trying again because second radiation treatments often have more side effects and are more dangerous. Still, Koyfman agreed to give him radiation again.
“There wasn’t much else,” he says. “Only radiation worked. And I said, ‘Well, if only radiation worked, but the regular radiation works for a short period of time why don’t we double down on radiation?’” Koyfman believed that stereotactic body radiation therapy, “high dose radiation to very small areas,” could benefit Agler.
“(It is) much more effective at eradicating the tumor in a shorter period of time,” he says. But it has to be extremely precise in head and neck cancers. “Perfect example is the spinal cord where if you damage even a half centimeter of cord, anywhere along the cord, you’re going to have paralysis,” Koyfman says.
“You have all these things, the carotid artery, the tongue, the throat, the eyes, it’s not like you can take out just a little bit of that and it’s no big deal.” After talking with Agler, he agreed. It felt like his only option because the mass grew so large.
“He had a tumor that was probably the size of a golf ball that went from a golf ball to a grapefruit in a matter of a couple weeks,” Koyfman says. “It was from the top of his nose all the way down his throat.” While the SBRT finally treated Agler's cancer, it created a hole where infection flourished.
After several courses of antibiotics including IV antibiotics, surgeons were able to go in treat the infection by removing dead tissue and repair the hole. “There was no cancer,” Koyfman says. “The radiation had cured the cancer.
It had created this secondary problem.” On October 1, 2020, Agler underwent a 19-hour surgery to remove the necrotic tissue and repair his throat. The time leading up to it was nervewracking.
“Aaron couldn’t open his mouth — and he still can’t,” Styer says. “We didn’t know what they were going to be able to do.” Doctors were able to remove the necrotic tissue and repair the hole.
Amazingly, Agler felt little pain after the lengthy procedure. “He had a pain pump,” Styer says. “He wasn’t pushing it.
He took no pain medication after that. It was Tylenol that he was taking, and he was fine.” The combination of radiation, chemotherapy and surgery created some damage.
The left side of Agler’s tongue and his left vocal cord are paralyzed. Doctors also removed his soft palate during surgery. That makes Agler’s speaking voice very soft and eating difficult and he relies on a liquid diet.
Agler currently has no evidence of disease but he's wary about saying that after going through so many recurrences. Still, he remains upbeat. “He was so positive through the whole thing and that changed everything,” Styer says.
“If you have a good attitude and a positive attitude that’s half the battle.” The couple wanted to share their story to encourage others. “Never give up.
Honestly, just keep pushing. You need to have a good relationship with your doctors. They need to understand you,” Styer says.
“His doctors pushed so hard to try and save him and that’s why he’s here.” Meghan Holohan is a digital health reporter for TODAY.com and covers patient-centered stories, women’s health, disability and rare diseases.
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