Overview

Pleuropulmonary blastoma (PPB) is a rare type of childhood lung tumor that begins in the chest either in the lung tissue itself (pulmonary) or in the tissue that covers the lungs and the inside of the chest cavity (pleura). A tumor begins when normal cells begin to change and grow uncontrollably. A tumor can be benign (noncancerous) or malignant (cancerous, meaning it can spread to other parts of the body).

There are four types of PPB:

• Type I is made up of cysts (air pockets) with early evidence that it is cancerous, but it is difficult to diagnose and may not be found in this early form. It occurs in very young children and has a better chance of being successfully treated than Types II and III (see below).
  
  
• Type Ir (the “r” stands for regressing) is similar to Type I, but it does not have cancerous cells.
  
  
• Types II and III generally occur in children ages two to four. Types II and III are cancerous tumors and require intensive chemotherapy.

More details on the different types of PPB are explained in other sections.

Statistics

PPB occurs most often in children younger than four, sometimes in children ages four to eight, and very rarely in older children, teenagers, and adults. PPB occurs in boys and girls approximately equally.

The overall relative survival rate (percentage of people who survive after the cancer is detected, excluding those who die from other diseases) of people with Type I PPB is around 83%, but may be higher or lower depending on several factors, and ranges from 45% to 50% for Types II and III.

Because PPB is so rare, these estimates are based on data collected by the International PPB Registry. Only 10 to 20 people are diagnosed with PPB each year in the United States.

Cancer survival statistics should be interpreted with caution. It is not possible to tell a person how long he or she will live with PPB. Survival statistics may not represent advances made in the treatment or diagnosis of this cancer.

Find out more about basic cancer terms used in this section.