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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.
Tests to diagnose pleuropulmonary blastoma
If your child has symptoms that suggest a pleuropulmonary blastoma, the doctor will need to find out if these are due to cancer or another problem. The doctor will ask when the symptoms started and how often your child has been having them. They will also ask about your child's personal and family medical history and do a physical exam. Depending on these results, they may recommend other tests. If your child is diagnosed with pleuropulmonary blastoma, the results of these tests will help you and your child's doctor plan treatment.
The tests used to diagnose pleuropulmonary blastoma may include:
Chest x-ray
An x-ray is a type of radiation that can go through the body and make pictures. A chest x-ray is one that makes pictures of the organs and bones inside the chest.
CT scan (CAT scan)
A CT scan uses a computer linked to an x-ray machine to make a series of detailed pictures of areas inside the body, such as the chest and abdomen. The pictures are taken from different angles and are used to create 3-D views of tissues and organs. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. Learn more about Computed Tomography (CT) Scans and Cancer.
Computed tomography (CT) scan. The child lies on a table that slides through the CT scanner, which takes a series of detailed x-ray pictures of areas inside the body.
PET scan (positron emission tomography scan)
A PET scan uses a small amount of radioactive sugar (also called radioactive glucose) injected into a vein. Then the PET scanner rotates around the body to make detailed, computerized pictures of areas inside the body where the glucose is taken up. Because cancer cells often take up more glucose than normal cells, the pictures can be used to find cancer cells in the body.
Positron emission tomography (PET) scan. The child lies on a table that slides through the PET scanner. The head rest and white strap help the child lie still. A small amount of radioactive glucose (sugar) is injected into the child's vein, and a scanner makes a picture of where the glucose is being used in the body. Cancer cells show up brighter in the picture because they take up more glucose than normal cells do.
Magnetic resonance imaging (MRI)
MRI uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas of the body, such as the head. This procedure is also called nuclear magnetic resonance imaging (NMRI).
Echocardiogram
Echocardiogram uses high-energy sound waves (ultrasound) that bounce off the heart and nearby tissues or organs and make echoes. A moving picture is made of the heart and heart valves as blood is pumped through the heart.
Ultrasound exam
Ultrasound exam uses high-energy sound waves (ultrasound) that bounce off internal tissues or organs and make echoes. The echoes form a picture of body tissues called a sonogram.
Bronchoscopy
Bronchoscopy is 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
Thoracoscopy is a type of surgery used to look inside the chest for abnormal areas. For this surgery, a thoracoscope is inserted into the chest through a cut made between two ribs. 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. Sometimes part of the esophagus or lung is also removed. A thoracotomy may be done if the thoracoscope cannot reach certain tissues, organs, or lymph nodes. In this procedure, a larger incision is made between the ribs and the chest is opened.
DICER1genetic testing
DICER1 genetic testing examines a sample of blood or saliva for changes in the DICER1 gene.
Immunohistochemistry
An immunohistochemistry test uses antibodies to check for certain antigens (markers) in a sample of a patient's tissue. The antibodies are usually linked to an enzyme or a fluorescent dye. After the antibodies bind to the antigen in the sample of the patient's tissue, the enzyme or dye is activated, and the antigen can then be seen under a microscope. This test is used to help diagnose cancer and to help tell one type of cancer from another type.
Types of treatment for pleuropulmonary blastoma
Who treats children with pleuropulmonary blastoma?
A pediatric oncologist, a doctor who specializes in treating children with cancer, oversees treatment of pleuropulmonary blastoma. The pediatric oncologist works with other health care providers who are experts in treating children with cancer and also specialize in certain areas of medicine. Other specialists may include:
- pediatrician
- pediatric surgeon
- pathologist
- pediatric nurse specialist
- social worker
- rehabilitation specialist
- psychologist
- child-life specialist
- fertility specialist
There are different types of treatment for children with pleuropulmonary blastoma. You and your child's care team will work together to decide treatment. Many factors will be considered, such as where the cancer is located and your child's age and overall health.
Your child's treatment plan will include information about the tumor, the goals of treatment, treatment options, and the possible side effects. It will be helpful to talk with your child's care team before treatment begins about what to expect. For help every step of the way, see our booklet, Children with Cancer: A Guide for Parents.
Types of treatment your child might have include:
Surgery
For all types of pleuropulmonary blastoma, surgery is done to remove the whole lobe of the lung with cancer. Chemotherapy may be given to shrink the tumor before surgery for patients with Types II or III pleuropulmonary blastoma. 
Lobectomy. A lobe of the lung is removed.
Chemotherapy
Chemotherapy (also called chemo) uses drugs to stop the growth of cancer cells. Chemotherapy either kills the cancer cells or stops them from dividing. Chemotherapy may be given alone or with other types of treatment. Chemotherapy for pleuropulmonary blastoma is injected into a vein. When given this way, the drugs enter the bloodstream and can reach cancer cells throughout the body.
Chemotherapy drugs used alone or in combination to treat pleuropulmonary blastoma include:
- dactinomycin
- doxorubicin
- ifosfamide
- vincristine
Other chemotherapy drugs not listed here may also be used.
Learn more about Chemotherapy to Treat Cancer.
Clinical trials
For some children, joining a clinical trial may be an option. There are different types of clinical trials for childhood cancer. For example, a treatment trial tests new treatments or new ways of using current treatments. Supportive care and palliative care trials look at ways to improve quality of life, especially for those who have side effects from cancer and its treatment.
You can use the clinical trial search to find NCI-supported cancer clinical trials accepting participants. The search allows you to filter trials based on the type of cancer, your child's age, and where the trials are being done. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website.
Learn more about clinical trials, including how to find and join one, at Clinical Trials Information for Patients and Caregivers.
Treatment of pleuropulmonary blastoma
Treatment of pleuropulmonary blastoma may include:
- For Type I and Type Ir pleuropulmonary blastoma, surgery is done to remove the whole lobe of the lung with cancer.
- For Type II and Type III pleuropulmonary blastoma, surgery is done to remove the whole lobe of the lung with cancer. Chemotherapy may be given before surgery to shrink the tumor.
If the cancer comes back after treatment, your child's doctor will talk with you about what to expect and possible next steps. There might be treatment options that may shrink the cancer or control its growth. If there are no treatments, your child can receive care to control symptoms from cancer so they can be as comfortable as possible.
