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

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 find and diagnose in this early form. It occurs in very young children (on average, 10 months old) 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 three 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.

Find out more about basic terms used in this section. [2]

Looking for More of an Overview?

If you would like additional introductory information, explore this related item on Cancer.Net:

• Cancer.Net Patient Education Video [3]:  View a short video led by an ASCO expert in childhood cancer that provides basic information and areas of research.

Or, choose “Next” (below, right) to continue reading this detailed section. To select a specific topic within this section, use the icon panel located on the right side of your screen.