Pleuropulmonary Blastoma - Childhood: Stages

Approved by the Cancer.Net Editorial Board, 06/2021

ON THIS PAGE: You will learn about how doctors describe a tumor’s growth or spread. This is called the stage. To see other pages, use the menu.

Staging is a way of describing where a tumor is located, if or where it has spread, and whether it is affecting other parts of the body. Doctors use diagnostic tests to find out the tumor's stage, so staging may not be complete until all of the tests are finished. Knowing the stage helps the doctor recommend what kind of treatment is best and can help predict a patient's prognosis, which is the chance of recovery. There are different stage descriptions for different types of tumors.

The following criteria help doctors describe PPB:

Type I: Type I PPB generally occurs in children younger than 1 and is made up of mostly cysts with almost no small, solid tumors, called nodules. A thin layer of the wall of the cyst may appear cancerous. Type I PPB usually has the best prognosis of the different types.

Type Ir: Type Ir is made up of cysts that do not contain cancerous cells.

Type II: Type II PPB contains both cysts and cancerous nodules. It occurs most commonly in children around 3 years old.

Type III: Type III PPB is a solid cancerous tumor. It occurs most often in children around 4 years old.

For Types II and III, a bone scan and MRI (see Diagnosis) may be necessary to detect the stage.

Advanced: Advanced PPB is when the tumor has spread beyond where it started. Often this means the tumor has spread within the chest cavity or to the diaphragm, the thin muscle under the lungs and heart that separates the chest from the abdomen. PPB can also spread through the bloodstream to other organs, including the bones, liver, and brain.

Recurrent: Recurrent cancer is cancer that has come back after treatment. If the cancer does return, there will be another round of tests to learn about the extent of the recurrence. These tests and scans are often similar to those done at the time of the original diagnosis.

Sources: Dehner LP: Pleuropulmonary blastoma is THE pulmonary blastoma of childhood, Seminars in Diagnostic Pathology, 11:144-51, 1994, and The International PPB Registry.

Information about the tumor’s stage will help the doctor recommend a specific treatment plan. The next section in this guide is Types of Treatment. Use the menu to choose a different section to read in this guide.