Genetic source of rare childhood cancer found; gene is implicated in other cancers
The search for the cause of an inherited form of a rare, aggressive childhood lung cancer has uncovered important information about how the cancer develops and potentially sheds light on the development of other cancers.
The finding by researchers at Washington University School of Medicine in St. Louis, Children's National Medical Center in Washington, D.C., the International Pleuropulmonary Blastoma Registry at Children's Hospitals and Clinics of Minnesota, and other collaborating institutions adds the final link to the chain connecting the gene DICER1 to cancer development — something that had been suspected but until now not definitively demonstrated.
The study shows that some children with the rare cancer pleuropulmonary blastoma (PPB) are born with a deleterious mutation in DICER1.
"PPB is the first malignancy found to be directly associated with inherited DICER1 mutations, making the cancer an important model for understanding how mutations and loss of DICER1 function lead to cancer," says lead author D. Ashley Hill, M.D., chief of pathology at Children's National Medical Center. "Additionally, we now believe that PPB tumors arise from an unusual mechanism in which cells carrying mutations induce nearby cells to become cancerous without becoming cancerous themselves."
The researchers found that all the children studied with PPB carried damaging mutations in one of their DICER1 genes, giving them one functional and one nonfunctional DICER1 gene in all their body's cells. The researchers indicate that PPB lung tumors probably originate when one or more cells in the lung
They discovered that loss of DICER1 protein specifically in lung airway cells appears to deregulate signals to nearby cells and somehow causes those cells to transform into malignant cells. However, the cells with the loss of DICER1 do not progress to malignancy.
This is important because finding out how this gene is altered in cancer could possibly explain other types of cancer.
http://mednews.wustl.edu/news/page/normal/14011.html
The finding by researchers at Washington University School of Medicine in St. Louis, Children's National Medical Center in Washington, D.C., the International Pleuropulmonary Blastoma Registry at Children's Hospitals and Clinics of Minnesota, and other collaborating institutions adds the final link to the chain connecting the gene DICER1 to cancer development — something that had been suspected but until now not definitively demonstrated.
The study shows that some children with the rare cancer pleuropulmonary blastoma (PPB) are born with a deleterious mutation in DICER1.
"PPB is the first malignancy found to be directly associated with inherited DICER1 mutations, making the cancer an important model for understanding how mutations and loss of DICER1 function lead to cancer," says lead author D. Ashley Hill, M.D., chief of pathology at Children's National Medical Center. "Additionally, we now believe that PPB tumors arise from an unusual mechanism in which cells carrying mutations induce nearby cells to become cancerous without becoming cancerous themselves."
The researchers found that all the children studied with PPB carried damaging mutations in one of their DICER1 genes, giving them one functional and one nonfunctional DICER1 gene in all their body's cells. The researchers indicate that PPB lung tumors probably originate when one or more cells in the lung
They discovered that loss of DICER1 protein specifically in lung airway cells appears to deregulate signals to nearby cells and somehow causes those cells to transform into malignant cells. However, the cells with the loss of DICER1 do not progress to malignancy.
This is important because finding out how this gene is altered in cancer could possibly explain other types of cancer.
http://mednews.wustl.edu/news/page/normal/14011.html
posted by Amber Thrapp at 4:44 PM
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