Share Print

Grant Will Help Researcher Seek Causes of Pediatric Lung Tumor

Aug. 23, 2007 – D. Ashley Hill, MD, assistant professor of pathology and immunology at Washington Unversity School of Medicine and a research member of the Siteman Cancer Center at Barnes-Jewish Hospital and Washington University School of Medicine in St. Louis, will receive a two-year grant from The Hope Street Kids, a program that supports and promotes research into pediatric tumors.

The program will provide $70,000 over the next two years to support Hill's search for the genetic causes of pleuropulmonary blastoma (PPB), a rare childhood lung tumor originally identified by Hill's mentor at Washington University, Louis P. "Pepper" Dehner, MD, professor of pathology and immunology and of pathology in pediatrics.

About half of the children affected by PPB do not survive. The condition leads to formation of lung tumors in the first five years of life and can also cause kidney cancer and other tumors. Early diagnosis and aggressive treatment improve chances of survival up to 80 to 90 percent.

But early diagnosis can be challenging. PPB is rare, and early stage PPB cancers sometimes resemble other more benign tumors.

"We are making some progress on this front," Hill says. "In 1995, only 14 percent of children with PPB were picked up in the early stage. Today in 2007, nearly 40 percent of children diagnosed with PPB have the early form."

But that still leaves 60 percent of children with PPB that isn't caught as early as physicians would like, Hill notes. A fuller understanding of the genetic basis of PPB may both help clinicians develop better screens for the condition and more effective treatments for the disease when it isn't diagnosed early.

Finding the mutations that cause PPB is sure to be challenging: PPB is a rare condition, with only 300 cases in the past 16 years, and about 15 new diagnoses each year. Many of those patients are enrolled in a registry, but to effectively seek the genetic roots of PPB, scientists needed to assemble a much more detailed collection of information not just on patients but on their extended family members as well.

"PPB sits at the crux of a group of childhood tumors that include rhabdomyosarcomaWilms' tumor and neuroblastoma," Hill notes. "So the hope is that if we can learn more about the origins of PPB, we will not only have the potential for better diagnosis and treatment of PPB, we may also shed some light on these much more common tumors of childhood."