Childhood Pleuropulmonary Blastoma Treatment (PDQ®): Treatment - Patient Information [NCI]

General Information About Pleuropulmonary Blastoma Treatment

Pleuropulmonary blastoma is a type of childhood lung cancer that forms in the tissues of the lung and pleura or the organs between the lungs.

Pleuropulmonary blastomas form in the tissue of the lung and pleura (tissue that covers the lungs and lines the inside of the chest). They 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).

In most cases, pleuropulmonary blastomas are linked to a certain change in the DICER1gene.

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 three types of pleuropulmonary blastoma.

The types of pleuropulmonary blastoma include the following:

• Type I tumors are cyst -like tumors in the lung. They are most common in children aged 2 years and younger and have a good chance of recovery. Type Ir tumors are Type I tumors that have regressed (gotten smaller) or have not grown or spread. After treatment, a Type I tumor may recur as a Type II or III tumor.
• Type II tumors are cyst-like with some solid parts. These tumors sometimes spread to the brain or other parts of the body.
• Type III tumors are solid tumors. These tumors often spread to the brain or other parts of the body.

Having a certain change in the DICER1 gene increases the risk of pleuropulmonary blastoma.

Anything that increases your chance of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn't mean that you will not get cancer. Talk with your child's doctor if you think your child may be at risk.

Risk factors for pleuropulmonary blastoma include the following:

• Having a certain change in the DICER1 gene.
• Having a family history of DICER1 syndrome.

Signs and symptoms of pleuropulmonary blastoma include trouble breathing and lung infections.

These and other signs and symptoms may be caused by pleuropulmonary blastoma or by other conditions.

Check with your child's doctor if your child has any of the following:

• A cough that doesn't go away.
• Trouble breathing.
• Fever.
• Lung infections, such as pneumonia.
• Pain in the chest or abdomen.
• Loss of appetite.
• Weight loss for no known reason.

Tests that examine the chest are used to diagnose pleuropulmonary blastoma.

The following tests and procedures may be used:

• Physical exam and health history: An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patient's health habits and past illnesses and treatments will also be taken.
• Chest x-ray: An x-ray of the organs and bones inside the chest. An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body.
• CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, such as the chest and abdomen, taken from different angles. The pictures are made by a computer linked to an x-ray machine. 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.
  
  Computed tomography (CT) scan of the abdomen. The child lies on a table that slides through the CT scanner, which takes x-ray pictures of the inside of the abdomen.
• PET scan (positron emission tomography scan): A procedure to find malignant tumor cells in the body. A small amount of radioactive glucose (sugar) is injected into a vein. The PET scanner rotates around the body and makes a picture of where glucose is being used in the body. Malignant tumor cells show up brighter in the picture because they are more active and take up more glucose than normal cells do.
• MRI (magnetic resonance imaging): A procedure that 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).
• Bone scan: A procedure to check if there are rapidly dividing cells, such as cancer cells, in the bone. A very small amount of radioactive material is injected into a vein and travels through the bloodstream. The radioactive material collects in the bones with cancer and is detected by a scanner.
  
  Bone scan. A small amount of radioactive material is injected into the child's vein and travels through the blood. The radioactive material collects in the bones. As the child lies on a table that slides under the scanner, the radioactive material is detected and images are made on a computer screen.
• 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.

Certain factors affect prognosis (chance of recovery).

Prognosis depends on:

• The type of pleuropulmonary blastoma.
• Whether the cancer has spread to other parts of the body at the time of diagnosis.
• Whether the cancer was completely removed by surgery.

Stages of Pleuropulmonary Blastoma

After pleuropulmonary blastoma has been diagnosed, tests are done to find out if cancer cells have spread to nearby areas or to other parts of the body.

The process used to find out if pleuropulmonary blastoma has spread to tissues in the chest or to other parts of the body is called staging. The information gathered from the staging process is used to plan treatment. The results of the tests and procedures used to diagnose cancer are often also used to stage the disease.

Sometimes childhood pleuropulmonary blastoma recurs (comes back) after treatment.

There are three ways that cancer spreads in the body.

Cancer can spread through tissue, the lymph system, and the blood:

• Tissue. The cancer spreads from where it began by growing into nearby areas.
• Lymph system. The cancer spreads from where it began by getting into the lymph system. The cancer travels through the lymph vessels to other parts of the body.
• Blood. The cancer spreads from where it began by getting into the blood. The cancer travels through the blood vessels to other parts of the body.

Cancer may spread from where it began to other parts of the body.

When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood.

• Lymph system. The cancer gets into the lymph system, travels through the lymph vessels, and forms a tumor (metastatic tumor) in another part of the body.
• Blood. The cancer gets into the blood, travels through the blood vessels, and forms a tumor (metastatic tumor) in another part of the body.

The metastatic tumor is the same type of cancer as the primary tumor. For example, if pleuropulmonary blastoma spreads to the brain, the cancer cells in the brain are actually pleuropulmonary blastoma cells. The disease is metastatic pleuropulmonary blastoma, not brain cancer.

Treatment Option Overview

There are different types of treatment for children with pleuropulmonary blastoma.

Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment.

Because cancer in children is rare, taking part in a clinical trial should be considered. Some clinical trials are open only to patients who have not started treatment.

Children with pleuropulmonary blastoma should have their treatment planned by a team of doctors who are experts in treating childhood cancer.

Treatment will be overseen by a pediatric oncologist, a doctor who specializes in treating children with cancer. The pediatric oncologist works with other pediatric health professionals who are experts in treating children with cancer and who specialize in certain areas of medicine. This may include the following specialists and others:

• Pediatrician.
• Pediatric surgeon.
• Pathologist.
• Pediatric nurse specialist.
• Social worker.
• Rehabilitation specialist.
• Psychologist.
• Child-life specialist.

Two types of standard treatment are used for pleuropulmonary blastoma:

Surgery

Surgery to remove the whole lobe of the lung with cancer is used to treat pleuropulmonary blastoma.

Lobectomy. A lobe of the lung is removed.

Chemotherapy may be given before surgery to remove the tumor. When given before surgery, chemotherapy will shrink the tumor and reduce the amount of tissue that needs to be removed during surgery. Treatment given before surgery is called preoperative therapy or neoadjuvant therapy.

Chemotherapy

Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy).

New types of treatment are being tested in clinical trials.

This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website.

Targeted therapy

Targeted therapy is a type of treatment that uses drugs or other substances to attack cancer cells. Targeted therapies usually cause less harm to normal cells than chemotherapy or radiation therapy do.

Targeted therapy is being studied for the treatment of pleuropulmonary blastoma that has recurred (come back).

Treatment for pleuropulmonary blastoma may cause side effects.

For information about side effects that begin during treatment for cancer, see our Side Effects page.

Side effects from cancer treatment that begin after treatment and continue for months or years are called late effects. Late effects of cancer treatment may include:

• Physical problems.
• Changes in mood, feelings, thinking, learning, or memory.
• Second cancers (new types of cancer) or other conditions.

Some late effects may be treated or controlled. It is important to talk with your child's doctors about the possible late effects caused by some treatments.

Patients may want to think about taking part in a clinical trial.

For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment.

Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment.

Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward.

Patients can enter clinical trials before, during, or after starting their cancer treatment.

Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment.

Clinical trials are taking place in many parts of the country. Information about clinical trials supported by NCI can be found on NCI's clinical trials search webpage. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website.

Follow-up tests may be needed.

Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests.

Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your child's condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups.

Treatment of Pleuropulmonary Blastoma

For information about the treatments listed below, see the Treatment Option Overview section.

Treatment of newly diagnosed pleuropulmonary blastoma may include the following:

• Surgery to remove the whole lobe of the lung the cancer is in, for Type I and Type Ir pleuropulmonary blastoma.
• Surgery to remove the whole lobe of the lung the cancer is in, with or without chemotherapy, for Type II and Type III pleuropulmonary blastoma.

Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available.

Treatment of Recurrent Pleuropulmonary Blastoma

For information about the treatments listed below, see the Treatment Option Overview section.

Treatment of recurrent pleuropulmonary blastoma in children may include the following:

• 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.

To Learn More About Pleuropulmonary Blastoma

For more information from the National Cancer Institute about pleuropulmonary blastoma, see the following:

• Lung Cancer Home Page
• Computed Tomography (CT) Scans and Cancer
• Targeted Cancer Therapies

For more childhood cancer information and other general cancer resources, see the following:

• About Cancer
• Childhood Cancers
• CureSearch for Children's Cancer
• Late Effects of Treatment for Childhood Cancer
• Adolescents and Young Adults with Cancer
• Children with Cancer: A Guide for Parents
• Cancer in Children and Adolescents
• Staging
• Coping with Cancer
• Questions to Ask Your Doctor about Cancer
• For Survivors and Caregivers

About This PDQ Summary

About PDQ

Physician Data Query (PDQ) is the National Cancer Institute's (NCI's) comprehensive cancer information database. The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish.

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This PDQ cancer information summary has current information about the treatment of childhood pleuropulmonary blastoma. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care.

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Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary ("Updated") is the date of the most recent change.

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Clinical Trial Information

A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard." Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.

Clinical trials can be found online at NCI's website. For more information, call the Cancer Information Service (CIS), NCI's contact center, at 1-800-4-CANCER (1-800-422-6237).

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PDQ® Pediatric Treatment Editorial Board. PDQ Childhood Pleuropulmonary Blastoma Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/lung/patient/child-pleuropulmonary-blastoma-treatment-pdq. Accessed <MM/DD/YYYY>.

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Last Revised: 2021-04-15

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