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"They saved our son. How can thanks ever be enough?"
—Dorie Rossell, Evan's mom
Illinois boy's rare and aggressive tumor challenges specialists at University of Iowa Children's Hospital
The news was so unexpected that Dorie Rossell reacted in angry disbelief.
Doctors in Peoria, Illinois, had just diagnosed her 2-year-old son, Evan, with a tumor of the jaw that was probably cancerous.
"It was like someone punched me in the stomach," she recalls. "They had to be wrong. A child does not get cancer from falling and hitting his chin on a coffee table!"
Yet that is exactly what seemed to be happening. Evan had slipped on a rug while running, striking his chin, creating a bump on the lower left side of his jaw.
The bump grew to the size of a small apple and would not go away.
Dorie and her husband, Greg, took Evan first to an orthopaedic specialist to see if his jaw was broken, then to a pediatrician who deferred to a dentist who was concerned that Evan had injured a saliva gland. Every test proved negative.
By February 2005, Evan was visiting the Peoria hospital, located near the family's hometown of Knoxville. While Dorie instinctively reacted in anger to the diagnosis, the Rossells felt better when a biopsy showed that the mass was non-malignant. Yes, they reasoned, surgery would be needed but the non-malignant part seemed encouraging.
Their optimism turned out to be premature. This reality set in after a Peoria surgeon, noting the rarity of this type of tumor—an aggressive fibromatosis or desmoid tumor—referred them to the specialists at University of Iowa Children's Hospital, located at University of Iowa Hospitals and Clinics.
Pediatric otolaryngologist Richard J. Smith, MD, who specializes in vascular anomalies, explained that the origin of aggressive fibromatosis tumors is unknown and the recurrence rate is high. "Unfortunately, they are extremely difficult to remove," he said.
Indeed, surgery would place at risk nerves that control taste and the muscle structure of the face. If the tumor recurred, more facial tissue would eventually have to be removed. In a worst case scenario, Evan's life could even be at risk.
In addition to Smith, Evan's multidisciplinary physician team included pediatric oncologist Raymond Tannous, MD, reconstructive surgery specialist Gerry Funk, MD, and maxillofacial surgery specialist Richard Burton, DDS. Together, they devised an extensive treatment plan that included chemotherapy as a prelude to reconstructive surgery or, in a best case scenario, no surgery at all.
Unfortunately, Evan's tumor was unresponsive to chemotherapy. Despite the risks, reconstructive surgery was the best remaining option.
During a highly complex 6-hour procedure performed in June 2005, the team removed the tumor in one whole piece. A temporary titanium plate was used to spare a small part of Evan's jaw so he could retain his baby teeth and hopefully grow healthy adult teeth later.
Smith says the team is confident that the entire tumor was removed, and pleased that his appearance is virtually normal and that he is showing evidence of adult teeth buds.
"The tumor has not recurred," he says. "So that's very encouraging. He still requires continuing care his jaw will need to be monitored through his teen-age years."
Dorie says she and her husband couldn't be happier with the quality of care and compassion they received at University of Iowa Children's Hospital.
"We count our blessings every day," she says. "Hopefully, with many prayers and the expertise of our doctors, this story will have a happy ending."
—Michael Sondergard
A tall, tall task
The University of Iowa Children's Hospital experts who removed Evan's aggressive tumor faced a complex and risky challenge. Like scar tissue, the tumor lacked clear borders between healthy and unhealthy tissue. In cases where these types of tumor appear on an arm or leg, amputation becomes a possibility. The team's highly specialized expertise in sparing vital nerves and healthy tissue was critically important to Evan's care. |
