ON THIS PAGE: You will find information about the estimated number of children who will be diagnosed with pleuropulmonary blastoma (PPB) each year. You will also read general information on surviving the disease. Remember, survival rates depend on several factors, and no 2 people with a tumor are the same. Use the menu to see other pages.
Every person is different, with different factors influencing their risk of being diagnosed with this tumor and the chance of recovery after a diagnosis. It is important to talk with your doctor about any questions you have around the general statistics provided below and what they may mean for your child individually. The original sources for these statistics are provided at the bottom of this page.
How many children are diagnosed with PPB?
PPB is rare. PPB occurs most often in children younger than 7. PPB is rarely diagnosed in teenagers and adults. Type Ir PPB (see Introduction) may be found in people of any age. PPB occurs about equally in boys and girls.
What is the survival rate for children with PPB?
There are different types of statistics that can help doctors evaluate a person’s chance of recovery from PPB. These are called survival statistics.
It is important to remember that statistics on the survival rates for people with PPB are only an estimate. They cannot tell an individual person if the tumor will or will not shorten their life. Instead, these statistics describe trends in groups of people previously diagnosed with the same disease, including specific stages of the disease.
The overall survival rate tells you what percent of children live a certain amount of time after the cancer is found. Percent means how many out of 100. The overall survival rate for children with PPB is hard to estimate because the disease is rare. The 5-year overall survival rate for children with Type I PPB is 89%. For children with Type Ir PPB, the 5-year overall survival rate is 100%. The 5-year overall survival rate for children with Type II or Type III PPB ranges from 53% to 71%. Type I PPB can recur, or come back, as Type II or III PPB.
The survival rates for children with PPB vary based on several factors. These include the stage of tumor, a person’s age and general health, and how well the treatment plan works. Talk with your doctor about your child’s prognosis, or chance of recovery, and what individual factors it is based on.
Estimates may not reflect the results of advancements in how PBB in children is diagnosed or treated. Talk with your child’s doctor if you have any questions about this information. Learn more about understanding statistics.
Statistics adapted from the websites of the International Pleuropulmonary Blastoma/DICER1 Registry, the National Organization for Rare Disorders, and the National Cancer Institute. (All sources accessed March 2023.)
The next section in this guide is Risk Factors. It describes the factors that may increase the chance of developing PPB. Use the menu to choose a different section to read in this guide.