New Center Treats Childhood Eye Cancer

April 1, 2009 - Texas Medical Center News
By Ronda Wendler


As Angela Van Beveren gazed into her newborn daughter’s eyes, she noticed something odd.

Sometimes baby Leah’s eyes rotated downward where only the tops showed – a condition Angela and her husband jokingly named "sunset eyes." Several weeks later, Angela noticed Leah’s left eye had begun to glow, and a white growth shown through the pupil.

We thought she had a lazy eye, so we took her to the doctor," Angela said.

When the diagnosis came, Angela and her husband were stunned. Leah had retinoblastoma, a cancerous tumor in her left eye.

Only 350 retinoblastoma cases are diagnosed each year in North America. The condition occurs when cancer develops in the cells of the retina, the lining in the back of the eye that sends visual images to the brain. The disease affects children from birth to 5 years of age, and is rare beyond age 6.

"Cancer was the last thing we expected to hear," said Angela. "With a new baby, two toddlers and a husband headed for military duty overseas, I was overwhelmed."

Doctors at Texas Children’s Cancer Center treated Leah with chemotherapy. Initially she responded well to the treatment, but a few months later more tumors appeared. Doctors delivered a frightening prognosis.

"They said Leah had less than a 10 percent chance of survival unless they removed her eye," Angela said. "I told my husband I’d rather be measuring Leah for a prosthetic eye than a coffin."

To help children like Leah, doctors and scientists from four Texas Medical Center institutions have formed the Retinoblastoma Center of Houston, where they will treat patients and conduct research aimed at preventing and curing the disease.

The center is the only one of its kind in the southwest United States, and the only center in the country to offer gene therapy to children enrolled in clinical trials.

"Retinoblastoma is a debilitating disease that attacks the most vulnerable – infants and children," said Patricia Chevez-Barrios, M.D., research co-director of the center and director of ophthalmic pathology at The Methodist Hospital. "Our best treatments are not good enough because we often have to remove the child’s eye or give chemotherapy or radiation that can have harmful side effects in children."

The center’s goal, she said, is to cure the cancer and save the eyes.

Retinoblastoma is caused by a mutation, or change, in a gene that resides on chromosome 13. When the gene mutates, cells can turn cancerous. One or both eyes may be affected, and telltale signs include discoloration of the pupil, and crossed or misaligned eyes.

Nearly 95 percent of children with retinoblastoma in the United States are cured, but they may have to lose an eye to lose the cancer. Early detection is crucial, or the disease can be fatal.

"The optic nerve connects the eye to the brain," Chevez-Barrios explained. "We don’t want the cancer to get into the optic nerve, because it can serve as a bridge to carry the cancer to the brain."

Patients at the Retinoblastoma Center of Houston will be seen at Texas Children’s Cancer Center and at the Children’s Cancer Hospital at M.D. Anderson. Patients will have access to a special kind of radiation called proton therapy, which helps spare healthy tissue around the tumor and lessens the risk of secondary cancers.

The center will also offer genetic testing for family members, an important element because retinoblastoma is often hereditary.

The Methodist Hospital Research Institute will house the center’s tumor bank and analyze tissue samples taken from patients and families.

"Since this cancer is so rare, it’s important for us to come together as a team to share our expertise and bring the best care to all retinoblastoma patients," said Dan Gombos, M.D., clinical co-director of the center and associate professor of ophthalmology at M.D. Anderson Cancer Center.

Angela Van Beveren says her daughter is in expert hands. Leah, now 5, is cancer-free after Texas Children’s doctors removed her left eye when she was 14 months old. She returns to Texas Children’s from her home in San Antonio for a checkup twice a year.

"It’s a three-hour drive but I’d drive 300 hours to come here," Angela said. "These people saved my daughter’s life."

Leah wears a perfectly crafted prosthetic eye that offers no hint of her ordeal. It’s a "hero eye," Leah says, because without it she likely would not have lived.

In addition to Chevez-Barrios and Gombos, other center co-directors are Murali Chintagumpala, M.D., pediatric oncologist at Texas Children’s Cancer Center and professor of pediatrics at Baylor College of Medicine; Richard Hurwitz, M.D., translational research director at Texas Children’s Cancer Center and associate professor of pediatrics, ophthalmology, and molecular and cellular biology at Baylor; and Peter Zage, M.D., Ph.D., assistant professor of pediatrics at M.D. Anderson’s Children’s Cancer Hospital.

Learn more...

photo photo photo photo