Pleuropulmonary Blastoma - Childhood: Types of Treatment

Approved by the Cancer.Net Editorial Board, 06/2021

ON THIS PAGE: You will learn about the different types of treatments doctors use for children with PPB. Use the menu to see other pages.

In general, tumors in children are uncommon. This means it can be hard for doctors to plan treatments unless they know what has been most effective in other children. That is why more than 60% of children with cancer are treated as part of a clinical trial. A clinical trial is a research study that tests a new approach to treatment. The “standard of care” is the best treatments known based on research studies. Clinical trials may test such approaches as a new drug, a new combination of existing treatments, or new doses of current therapies. The health and safety of all children participating in clinical trials are closely monitored.

To take advantage of these newer treatments, children with PPB should be treated at a specialized treatment center. Doctors at these centers have extensive experience in treating children and have access to the latest research. A doctor who specializes in treating children with cancer is called a pediatric oncologist. If a pediatric cancer center is not nearby, general cancer centers sometimes have pediatric specialists who are able to be part of your child’s care.

How PPB is treated

In many cases, a team of doctors works with a child and the family to provide care. This is called a multidisciplinary team. Pediatric cancer centers often have extra support services for children and their families, such as child life specialists, dietitians, physical and occupational therapists, social workers, and counselors. Special activities and programs to help your child and family cope may also be available.

The common types of treatments used for PPB are described below. Treatment options and recommendations depend on several factors, including the type and stage of the tumor, possible side effects, and the patient’s preferences and overall health. Your child’s care plan also includes treatment for symptoms and side effects, an important part of cancer care.

Surgery and chemotherapy, and sometimes radiation therapy, are the main treatment options for PPB. If all of the tumor cannot be removed during surgery, chemotherapy and/or radiation therapy may be used as well.

Take time to learn about all of your child’s treatment options and be sure to ask questions about things that are unclear. Talk with your child’s doctor about the goals of each treatment and what your child can expect while receiving the treatment. These types of talks are called "shared decision-making." Shared decision-marking is when you and your child's doctors work together to choose treatments that fit the goals of your child's care. Shared decision-making is particularly important for PPB because there are different treatment options. Learn more about making treatment decisions.

Surgery

Surgery is the removal of the tumor and some surrounding healthy tissue during an operation. It is the main treatment for PPB. A surgical oncologist is a doctor who specializes in treating a tumor using surgery.

If the tumor is too large to be completely removed, or if it is not possible to completely remove the tumor, chemotherapy may be given before surgery to reduce the size of the tumor. This is called pre-operative or neoadjuvant chemotherapy. Chemotherapy and sometimes radiation therapy (see below) may also be given after surgery to destroy any remaining cells, called adjuvant therapy.

Before surgery, talk with your health care team about the possible side effects from the specific surgery that your child may experience and what recovery will be like. Learn more about the basics of cancer surgery.

Chemotherapy

Chemotherapy is the use of drugs to destroy tumor cells, usually by keeping the cancer cells from growing, dividing, and making more cells.

A chemotherapy regimen, or schedule, which is your child’s treatment schedule, usually consists of a specific number of cycles given over a set period of time. A patient may receive 1 drug at a time or a combination of different drugs given at the same time. Chemotherapy is often used to treat childhood tumors, and even children as young as newborn infants can be safely treated for life-threatening tumors with chemotherapy.

In some patients, Type I PPB is treated with surgery, and the role of chemotherapy after surgery is debated. Talk with your child’s doctor about whether it is recommended in your child’s case. Chemotherapy is always recommended for Types II and III PPB. The doctor may perform surgery first and then give chemotherapy after the surgery to destroy any remaining PPB cells. This is called adjuvant or post-operative chemotherapy. Sometimes, pre-operative chemotherapy is given.

Common drugs for Types II and III PPB include: cyclophosphamide (Neosar), vincristine (Vincasar) and dactinomycin (Cosmegen). Common treatment regimens for Types II and III include actinomycin D (IVADo), doxorubicin (Adriamycin), ifosfamide (Ifex), and vincristine (Vincasar).

The side effects of chemotherapy depend on the individual and the dose used, but they can include fatigue, risk of infection, nausea and vomiting, hair loss, loss of appetite, and diarrhea. These side effects usually go away after treatment is finished.

Learn more about the basics of chemotherapy.

Radiation therapy

Radiation therapy is the use of high-energy x-rays or other particles to destroy tumor cells. A doctor who specializes in giving radiation therapy to treat a tumor is called a radiation oncologist.

The most common type of radiation treatment is called external-beam radiation therapy, which is radiation given from a machine outside the body. When radiation treatment is given using implants, it is called internal radiation therapy or brachytherapy. A radiation therapy regimen, or schedule, usually consists of a specific number of treatments given over a set period of time.

Not every child with PPB receives radiation therapy. Radiation therapy may be recommended when the child’s health care team believes that there are still cancerous cells remaining after surgery and chemotherapy.

Side effects from radiation therapy may include fatigue, mild skin reactions, upset stomach, and loose bowel movements. Most side effects go away soon after treatment is finished. However, radiation therapy can also interfere with normal bone growth and is associated with development of secondary cancers. Talk with your child’s doctor about the possible short-term and long-term side effects before treatment begins. Learn more about the basics of radiation therapy.

Physical, emotional, and social effects of cancer

Cancer and its treatment cause physical symptoms and side effects, as well as emotional, social, and financial effects. Managing all of these effects is called palliative care or supportive care. It is an important part of your child’s care that is included along with treatments intended to slow, stop, or eliminate the cancer.

Palliative care focuses on improving how your child feels during treatment by managing symptoms and supporting patients and their families with other, non-medical needs. Any person, regardless of age or type and stage of cancer, may receive this type of care. And it often works best when it is started right after a cancer diagnosis. People who receive palliative care along with treatment for the cancer often have less severe symptoms, better quality of life, and report that they are more satisfied with treatment.

Palliative treatments vary widely and often include medication, nutritional changes, relaxation techniques, emotional and spiritual support, and other therapies. Your child may also receive palliative treatments similar to those meant to get rid of the cancer, such as chemotherapy, surgery, or radiation therapy.

Before treatment begins, talk with your child’s doctor about the goals of each treatment in the treatment plan being recommended. You should also talk about the possible side effects of the specific treatment plan and palliative care options. Many patients also benefit from talking with a social worker and participating in support groups. Ask your doctor about these resources, too.

During treatment, your child’s health care team may ask you to answer questions about your child’s symptoms and side effects and to describe each problem. Be sure to tell the health care team if your child is experiencing a problem. This helps the health care team treat any symptoms and side effects as quickly as possible. It can also help prevent more serious problems in the future.

Learn more about the importance of tracking side effects in another part of this guide. Learn more about palliative care in a separate section of this website.

Advanced PPB

Advanced PPB is when the tumor has spread beyond where it started, mostly within the chest cavity or to the diaphragm, the thin muscle under the lungs and heart that separates the chest from the abdomen. PPB can also spread through the bloodstream to other organs, especially the bones, liver, and brain.

Areas where PPB has spread are found by imaging tests, such as a CT scan, MRI scan, or bone scan. See the Diagnosis section for more information on these tests. If 1 area where PPB has spread is found on a scan, it is likely that the PPB has spread to other areas that cannot be seen on the scan. PPB that has spread is only rarely treated with surgery; other treatments will be recommended. Palliative care will also be important to help relieve symptoms and side effects.

A diagnosis of advanced PPB is stressful and difficult. Members of your family are encouraged to talk how your feel with your child's health care team. It may be also helpful to talk with other caregivers, such as a through a support group or peer support program.

Remission and the chance of recurrence

A remission is when PPB cannot be detected in the body and there are no symptoms. This may also be called having “no evidence of disease” or NED.

A remission may be temporary or permanent. This uncertainty causes many people to worry that the cancer will come back. While many remissions are permanent, it is important to talk with your child’s doctor about the possibility of the tumor returning. Understanding your child’s risk of recurrence and the treatment options may help you feel more prepared if the tumor does return. Learn more about coping with the fear of recurrence.

If the tumor returns after the original treatment, it is called a recurrent tumor. It may come back in the same place (called a local recurrence), nearby (regional recurrence), or in another place (distant recurrence).

When this occurs, a new cycle of testing will begin again to learn as much as possible about the recurrence. After testing is done, your child’s doctor will talk with you about treatment options. Often the treatment plan will include the treatments described above, such as surgery, chemotherapy, and radiation therapy, but they may be used in a different combination or given at a different pace. Your child’s doctor may also suggest clinical trials that are studying new ways to treat this type of recurrent tumor. Whichever treatment plan you choose, palliative care will be important for relieving symptoms and side effects.

Additional treatment depends on where and when the tumor recurred and how it was initially treated. Surgery may be used to remove a new tumor. Chemotherapy and/or radiation therapy may also be recommended. Bone marrow/stem cell transplant may also be a treatment option for some children.

People with recurrent cancer sometimes experience emotions such as disbelief or fear. You are encouraged to talk with the health care team about these feelings and ask about support services to help you cope. Learn more about dealing with cancer recurrence.

If treatment does not work

Although treatment is successful for many children with cancer, sometimes it is not. Sometimes, PPB cannot be cured or controlled. This diagnosis is stressful and may be difficult to discuss. However, it is important to have open and honest conversations with your child’s health care team to express your family’s feelings, preferences, and concerns. The health care team has special skills, experience, and knowledge to support patients and their families and is there to help.

Hospice care is designed to provide the best possible quality of life for people who are are expected to live less than 6 months. Parents and guardians are encouraged to talk with the health care team about hospice options, which include hospice care at home, a special hospice center, or other health care locations. Nursing care and special equipment can make staying at home a workable option for many families. Some children may be happier and more comfortable if they can attend school part-time or keep up other activities and social connections. The child’s health care team can help parents or guardians decide on an appropriate level of activity. Making sure a child is physically comfortable and free from pain is extremely important as part of end-of-life care. Learn more about caring for a terminally ill child and advanced cancer care planning.

The death of a child is an enormous tragedy, and families may need support to help them cope with the loss. Pediatric cancer centers often have professional staff and support groups to help with the process of grieving. Learn more on grieving the loss of a child.

The next section in this guide is About Clinical Trials. It offers more information about research studies that are focused on finding better ways to care for people with PPB. Use the menu to choose a different section to read in this guide.