Pleuropulmonary Blastoma - Childhood: Stages

Approved by the Cancer.Net Editorial Board, 10/2018

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

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 to decide 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 year and is made up of mostly cysts, with almost no small, solid tumors that are 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, and occurs most commonly in children around 3 years old.

Type III: Type III PPB is a solid cancerous tumor, and 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.

Recurrent: Recurrent cancer is cancer has come back after treatment. If there is a recurrence, the tumor may need to be staged again using the criteria above in a process called restaging.

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.