(June 26, 2009 - Insidermedicine) A genetic mutation underlying a rare form of cancer that develops in the lungs of children, called pleuropulmonary blastoma, has been discovered in a study published in the latest issue of Science.

Here is some information about PPB:

•    It occurs almost exclusively in children under the age of 7 to 8 years

•    About 25% of children with PPB have family members with this or a related condition

•    The inherited aspect of the condition suggests involvement of one or more genetic mutations

Researchers from Children's National Medical Center conducted genetic testing on 11 families affected by PPB. They also tested samples of lung and tumor tissue from PPB sufferers to understand how genetic mutations might be contributing to the development of this condition.

Among PPB-affected families, the researchers found mutations in a gene known as DICER1. While the protein produced by this gene could not be found in the tissue that lines the lungs, it was found in the cancerous tissue itself. It is believed DICER1 normally controls adjacent cell growth, but when mutated, adjacent cells can quickly start to grow, developing into cancers.

We had a chance to speak with Dr. Ashley Hill, the principal investigator of this study, who offered some further insight.

Today's research identifies a gene mutation that may cause changes in lung tissue, leading to the development of PPB. It also sheds more light on how cell to cell communication may result in the development of cancers.

For Insidermedicine in Depth, I'm Dr. Kelly Schweitzer.