Pleuropulmonary Blastoma - Childhood: Risk Factors

Approved by the Cancer.Net Editorial Board, 06/2022

ON THIS PAGE: You will find out more about the factors that increase the chance of developing pleuropulmonary blastoma (PPB). Use the menu to see other pages.

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.

About 70% of people with PPB have a genetic mutation of the DICER1 RNase-IIIb gene, which is a germline mutation. Germline mutations are inherited mutations that are passed down from one generation to the next in a family. About 80% of PPB patients inherited the DICER1 RNase-IIIb mutation from a parent.

The DICER1 syndrome 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.

For screening information for PPB and other cancers related to DICER1, see the Latest Research section of this guide.

The next section in this guide is Symptoms and Signs. It explains what changes or medical problems PPB can cause. Use the menu to choose a different section to read in this guide.