Symptoms and Signs of Pleuropulmonary Blastomas (PPBs) in Children

Medical Author:
Medically Reviewed on 9/21/2021

Doctor's Notes on Pleuropulmonary Blastomas (PPBs) in Children

Pleuropulmonary blastomas (PPBs) are a rare type of cancer that forms in the tissue of the lung and pleura (tissue that covers the lungs and lines the inside of the chest). PPBs can also form in the organs between the lungs including the heart, aorta, and pulmonary artery, or in the diaphragm. The three types of PPB are:

  • type I tumors that are cyst-like tumors in the lung that can usually be cured,
  • type II tumors that are cyst-like with some solid parts that sometimes spread to the brain, and
  • type III tumors which are solid tumors that often spread to the brain. 

Symptoms of pleuropulmonary blastomas include:

What Is the Treatment for Pleuropulmonary Blastomas (PPBs) in Children?

Type I PBBs are typically treated with surgical removal of the tumor. In some cases, chemotherapy may be given after surgery.

Type II and III tumors are usually treated with surgery followed by chemotherapy. If the tumor is too large to successfully remove during surgery, chemotherapy may be given before surgery to shrink the tumor. In some cases, radiation therapy may also be given after surgery and chemotherapy.

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REFERENCE:

Kasper, D.L., et al., eds. Harrison's Principles of Internal Medicine, 19th Ed. United States: McGraw-Hill Education, 2015.