Pleuropulmonary Blastoma - Childhood: Introduction

Approved by the Cancer.Net Editorial Board, 06/2022

ON THIS PAGE: You will find some basic information about this disease and the parts of the body it may affect. This is the first page of Cancer.Net’s Guide to Pleuropulmonary Blastoma. Use the menu to see other pages. Think of that menu as a roadmap for this entire guide.

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 pleura, also called the chest cavity.

A tumor begins when healthy cells change and grow out of control, forming a mass called a tumor. A tumor can be cancerous or benign. A cancerous tumor is malignant, meaning it can grow and spread to other parts of the body. A benign tumor means the tumor can grow but will not spread.

There are 4 types of PPB:

  • Type I is made up of air pockets called cysts with early evidence that it is cancerous. However, it is difficult to find and diagnose PPB in this early form. Type I PPB occurs in very young children, who on average are 10 months old. It has a better chance of being successfully treated, usually with surgery, than other types of PPB, such as Types II and III, described below.

  • Type Ir is similar to Type I, but it does not have cancerous cells and occurs in patients older than in Type I. The “r” stands for regressing.

  • Types II and III generally occur in children ages 3 to 4. Types II and III are cancerous tumors and require more intensive therapy, which includes chemotherapy and surgery.

Many patients with PPB have a mutation of the DICER1 RNase-IIIb gene. More details on the different types of PPB are explained in other sections of this guide, including more information about genetic factors in Risk Factors.

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The next section in this guide is Statistics. It helps explain the number of children who are diagnosed with pleuropulmonary blastoma and general survival rates. Use the menu to choose a different section to read in this guide.