A rare cancer diagnosis, and an even rarer treatment for 6-year-old Elin
Elin Hoechst was diagnosed with a rare cancer, in a rare location, when she was only 2 years old.
In 2019, a pediatrician in her hometown of Bismarck, North Dakota, discovered a tumor in Elin’s jaw and referred the family to M Health Fairview Masonic Children’s Hospital for care. There, Pediatric Oncologist Emily Greengard, MD, an associate professor at University of Minnesota Medical School, confirmed the diagnosis of Ewing sarcoma.
Ewing sarcoma, a type of bone cancer, affects just over 200 children and teens nationwide every year. It’s especially uncommon in children as young as Elin. Despite its rarity, Ewing sarcoma is a familiar foe for the pediatric cancer care team at our children’s hospital, which treats Ewing sarcoma patients from across the Midwest.
Elin needed an uncommon and innovative care plan due to her age and the tumor’s location on her jaw. Greengard and her team first treated the cancer through chemotherapy, then our surgeons completely removed the tumor and rebuilt Elin’s lower jawbone.
Luke Jakubowski, MD, a pediatric otolaryngologist and assistant professor at University of Minnesota Medical School, led the surgery. He and his team came up with an innovative two-step solution given how small Elin was at the time of her diagnosis. First, they removed Elin’s lower jawbone to completely remove her tumor. At the time, they replaced the bone with a 3D-printed reconstruction.
Two years later, Elin came back to our children’s hospital for fibula free flap surgery under the care of Sofia Lyford-Pike, MD, an M Health Fairview facial plastic and reconstructive surgeon. Our surgical team removed part of Elin’s fibula bone, reconstructed it, and reattached it to serve as her new jawbone – one that fit Elin after two years of growth, and has the potential to grow with her into the future.
Jaw pain leads to rare diagnosis
Anne Hoechst, Elin’s mother, was washing Elin’s face one night in July 2019 when Elin, then 2 years old, became visibly uncomfortable. In the following weeks, a few of Elin’s teeth fell out – a strange event given Elin’s age. Anne brought Elin to their pediatrician in North Dakota, where a CT scan revealed the tumor in her jaw.
Elin was referred to M Health Fairview Masonic Children’s Hospital for treatment, where Greengard and our pediatric oncology team started her on chemotherapy. In addition to chemotherapy, Greengard knew the existing tumor would have to be addressed, either through surgery or radiation.
“Early on, we had a lot of discussions with our radiation oncologists as well as our surgical team. We worked together to determine the best plan,” said Greengard. “In a child Elin’s age, we try to avoid radiation because she has a lot of growing left to do. When you use radiation, you impact the growth on that area of the body.”
Greengard and her team worked to shrink the tumor with chemotherapy ahead of Elin’s surgery and used chemotherapy afterward as well to ensure there were no cancer cells left.
3D-printed jaw serves as bridge to full reconstruction
Our surgical team needed to remove Elin’s entire lower jawbone, given her size and the tumor’s connection to the bone. At the time, Elin was too small for a fibula free flap – a jaw reconstruction where surgeons take part of the fibula bone from a patient’s leg and reconstruct it to use as a jawbone. Jakubowski and his team used a 3D-printed metal jawbone to take its place until Elin was old enough for the full reconstruction.
“We talked over her options. Surgery was aggressive, but it gave her the best long-term outlook,” said Jakubowski. “Our primary goal was to treat the cancer and we had to be aggressive.”
Two years later, Elin was big enough to have fibula free flap surgery. Fibula free flap is a rare surgery in adults, let alone in children. Lyford-Pike led the reconstruction and is one of few physicians with experience in pediatric microvascular surgery. She used 3D modeling to plan for the reconstruction, utilizing Elin’s CT scans to create custom guides and plates for forming her fibula bone into a new jaw.
“Elin’s treatment demonstrated the incredible cooperation between our specialists,” said Lyford-Pike, who is also an associate professor at University of Minnesota Medical School. “There was no prescription. We brought together a team with a wide breadth of experience in their specialties and thought outside the box to come up with the best solution for Elin.”
Lyford-Pike performed the reconstruction under a microscope, delicately reconnecting the blood vessels she’d taken from Elin’s leg (along with her fibula) to the blood vessels in Elin’s face. Lyford-Pike was assisted under the microscope by Ashok Jethwa, MD, a head and neck surgical oncologist and assistant professor at University of Minnesota Medical School. Like Lyford-Pike, Jethwa specializes in microvascular surgery.
New jaw that can grow with Elin
After surgery, Elin recovered in our pediatric intensive care unit (PICU) and met with our rehabilitation services team, including pediatric speech-language pathologists, to help her adjust to her new jaw.
“The next step in Elin's journey is to get dental implants for her lower teeth,” said Jakubowski, who needed to remove Elin’s bottom teeth during her first surgery. “As she continues to grow, we will likely need to grow her jaw through a surgery called distraction osteogenesis, which will preserve her jaw profile as she ages.”
Both Elin’s surgeries were done using minimally invasive methods. The solution not only removed the cancer, but it will also lead to fewer issues later as Elin ages because of reduced scarring and a new jaw that can grow with her.
“One of our concerns was, would she have ongoing restrictions after treatment?” said her mother, Anne. “Our surgeons said she can pretty much do anything with her new jaw, and she was running around the minute her leg was healed. She’s back to swimming and, at her most recent check-up, they cleared her to be in gymnastics this summer.”
Over the past three years, Greengard and her team have continued to see Elin at regular imaging appointments. This monitoring helps ensure that, if Elin’s cancer were to return, we could catch and treat it as quickly as possible. Elin is now cancer-free three years after chemotherapy and is able to come in less often – every six months – for imaging. At five years post-chemotherapy, Elin will transition to our long-term follow-up clinic.
With her new jaw, Elin is ready to take on the world – enjoying summer and starting first grade this fall.
