Two years cancer-free after surgery for rare retinoblastoma
Four-year-old Hazel Farke recently celebrated both her birthday and another important milestone: two years of being cancer-free.
Hazel was diagnosed as a toddler with retinoblastoma. Retinoblastoma is an incredibly rare eye cancer sometimes caused by a gene mutation that can be new or inherited, or that can seemingly appear out of nowhere. Hazel’s family had no experience with the disease when her mother, Melisa, noticed a strange glow in Hazel’s left eye. It showed only during specific times, such as when Hazel would look up from her highchair at the kitchen light.
When the eye stopped tracking correctly, Melisa and her husband, Kenny, thought Hazel may need glasses. They took her to their local optometrist in South Dakota, who noticed a larger problem and referred them to a pediatric ophthalmologist. That provider confirmed that Hazel likely had retinoblastoma and connected the family with Jill Anderson, MD, a pediatric ophthalmologist at M Health Fairview Masonic Children’s Hospital and assistant professor in the Department of Ophthalmology at University of Minnesota Medical School, who specializes in retinoblastoma and other serious eye conditions.
“When we first met Dr. Anderson, her team said something that stuck with me — that retinoblastoma is a rare disease, but not within these walls,” said Melisa. “They were so informative. She told us everything we needed to know, gave us all possible treatment options, and then let us make the decision.”
There are just over 300 cases of retinoblastoma annually in the United States, but our children’s hospital sees a significant number of children with this rare disease.
“It’s a complex disease that requires multidisciplinary care from both ophthalmologists and pediatric oncologists and in certain cases, interventional radiologists,” said Anderson. “Many kids are sent to us specifically because we have the team of multidisciplinary pediatric specialists necessary to provide treatments for this disease.”
Treatment tailored to each situation
Retinoblastoma can occur in one or both eyes and spread to other parts of the body. In Hazel’s case, it was only in her left eye and the cancer hadn’t spread.
There are several treatments for the disease, with options growing in recent years. It used to be standard to treat retinoblastoma with systemic chemotherapy once a month for six months, plus additional laser or freezing treatment in the eye. While this is still the best option in some cases, doctors are also now able to provide targeted chemotherapy when appropriate directly to the eye area through a catheter or injection, helping to minimize systemic side effects of treatment.
Another option is enucleation, or surgical removal of the affected eye, which ended up being the best course of treatment for Hazel. Her tumor had grown large enough to involve over two-thirds of her eye.
“Given the size and spread of the tumor, we would have had to double the amount of chemotherapy, and there was a good chance that she may still have needed her eye removed even after chemotherapy,” said Melisa. “Dr. Anderson laid out our options and let us make the decision.”
“The likelihood that we would have been able to save her eye was not very high,” said Anderson. “And there was even a lower chance that we’d be able to save any vision in the affected eye.”
Surgery designed to stop the cancer’s spread
Hazel had enucleation surgery in October 2020, leaving the hospital on the same day. It’s a common procedure but with special adaptations in the case of retinoblastoma. Anderson and her team are experienced in this surgery, which includes special steps for optimal cancer care.
“Ophthalmologists are trained to do standard enucleation surgery, and we do them most commonly for to relieve pain in painful blind eyes,” said Anderson. “With retinoblastoma, we’re trying to remove a very long segment of the optic nerve attached behind the eye, because that’s one of the ways that the cancer can spread outside the eye. We want to get everything out, so that no tumor cells are left behind.”
Following a check-up visit, the Farke family was able to return home to Sioux Falls the day after surgery.
“She was up the next day, happy and feeling good,” Melisa said. “I figured she would sleep on the drive back to South Dakota, but she was up coloring and playing in the car.”