What is pleuropulmonary blastoma?
Pleuropulmonary blastoma is a rare and aggressive type of childhood cancer that forms in the lungs, the tissue that covers the lungs, and the inside wall of the chest cavity called the pleura. It can also form in the heart, aorta, pulmonary artery (the blood vessel that takes blood from the heart to the lungs), and the diaphragm (the main breathing muscle below the lungs). Pleuropulmonary blastoma mainly occurs in younger children, typically before the age of 5 years.
Pleuropulmonary blastoma is a rare, fast-growing cancer that forms in the tissues of the lung and pleura (the thin layer of tissue that covers the lungs and lines the inside of the chest). It may also form in the organs between the lungs, including the heart, aorta, and pulmonary artery, or in the diaphragm (the main breathing muscle below the lungs). Pleuropulmonary blastoma usually occurs in infants and young children.
There are four types of pleuropulmonary blastoma:
- Type I tumors are cyst -like lung tumors. They are most common in children younger than 3 years and children have a good chance of recovery. After treatment, sometimes a Type I tumor may recur as a Type II or III tumor.
- Type Ir tumors are Type I tumors that have gotten smaller (regressed) or have not grown or spread.
- Type II tumors are cyst-like with some solid parts. They are found more often in children older than 2 years. Type II tumors sometimes spread to the brain or other parts of the body.
- Type III tumors are solid tumors. They are found more often in children older than 2 years. Type III tumors often spread to the brain or other parts of the body.