Pleuropulmonary Blastomas (PPBs) in Children
What Is Pleuropulmonary Blastoma?
Pleuropulmonary blastomas (PPBs) form 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 main breathing muscle below the lungs).
There are three types of PPB:
- Type I tumors are cyst -like tumors in the lung. They are most common in children aged 2 years and younger and can usually be cured. Type Ir tumors are Type I tumors that have gotten smaller or have not grown or spread.
- Type II tumors are cyst-like with some solid parts. These tumors sometimes spread to the brain.
- Type III tumors are solid tumors. These tumors often spread to the brain.
What Are the Risk Factors for Pleuropulmonary Blastoma in Children?
The risk of PPB is increased by the following:
- Having pleuropulmonary blastoma familial cancer syndrome.
- Having a certain change in the DICER1 gene.
What Are the Signs and Symptoms of Pleuropulmonary Blastoma in Children?
PPB may cause any of the following signs and symptoms. Check with your child’s doctor if your child has any of the following:
Other conditions that are not PPB may cause these same signs and symptoms.
How Are Pleuropulmonary Blastomas in Children Diagnosed?
Tests to diagnose and stage PPB may include the following:
- Physical exam and history.
- X-ray of the chest.
- CT scan.
- PET scan.
Other tests used to diagnose PPB include the following:
- Bronchoscopy: A procedure to look inside the trachea and large airways in the lung for abnormal areas. A bronchoscope is inserted through the nose or mouth into the trachea and lungs. A bronchoscope is a thin, tube-like instrument with a light and a lens for viewing. It may also have a tool to remove tissue samples, which are checked under a microscope for signs of cancer.
- Thoracoscopy: A surgical procedure to look at the organs inside the chest to check for abnormal areas. An incision (cut) is made between two ribs, and a thoracoscope is inserted into the chest. A thoracoscope is a thin, tube-like instrument with a light and a lens for viewing. It may also have a tool to remove tissue or lymph node samples, which are checked under a microscope for signs of cancer. In some cases, this procedure is used to remove part of the esophagus or lung. If the thoracoscope cannot reach certain tissues, organs, or lymph nodes, a thoracotomy may be done. In this procedure, a larger incision is made between the ribs and the chest is opened.
PPBs may spread or recur (come back) even after being removed by surgery.
What Is the Treatment and Prognosis for Pleuropulmonary Blastomas in Children?
Treatment of pleuropulmonary blastoma in children includes the following:
- Surgery to remove the whole lobe of the lung the tumor is in, with or without chemotherapy.
Treatment of recurrent pleuropulmonary blastoma in children may include the following:
- A clinical trial of targeted therapy using a monoclonal antibody.
- A clinical trial that checks a sample of the patient's tumor for certain gene changes. The type of targeted therapy that will be given to the patient depends on the type of gene change.
The prognosis (chance of recovery) depends on the following:
- The type of pleuropulmonary blastoma.
- Whether the tumor has spread to other parts of the body at the time of diagnosis.
- Whether the tumor was completely removed by surgery.
The website of the National Cancer Institute (https://www.cancer.gov)
Last updated Oct. 6, 2017
Medically Reviewed by a Doctor on 11/9/2017