Pleuropulmonary Blastoma - Childhood - Risk Factors

Approved by the Cancer.Net Editorial Board, 11/2014

ON THIS PAGE: You will find out more about the factors that increase the chance of developing this type of tumor. To see other pages, use the menu on the side of your screen.

A risk factor is anything that increases a person’s chance of developing a tumor. Although risk factors often influence the development of a tumor, most do not directly cause a tumor. Some people with several risk factors never develop a tumor, while others with no known risk factors do.

The cause of PPB is not known. Most often it develops randomly. For most children with PPB, there may have been no personal or family history of the disease before the PPB occurred; in other words, there was no reason to suspect that the child would develop PPB. Most family members of children with PPB are healthy. The following factors may raise a person’s risk of developing PPB.

Cysts. Early stages of PPB appear as cysts. However, most children with lung cysts do not have PPB.

Family history/genetics. Many patients with PPB have a mutation of the DICER1 RNase-IIIb gene. The DICER1 syndrome is also known as Pleuropulmonary Blastoma Family Tumor and Dysplasia syndrome. PPB does not necessarily occur in all families with this gene mutation. This gene mutation is also associated with other cancers such as cystic nephroma, cervical rhabdomyosarcoma, nasal chondromesenchymal hamartoma, ovarian stromal tumors nodular thyroid hyperplasia, ciliary body medulloepithelioma and pineoblastoma.

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