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Pleuropulmonary Blastoma - Childhood - Overview

This section has been reviewed and approved by the Cancer.Net Editorial Board, 11/2014

ON THIS PAGE: You will find some basic information about this disease and the parts of the body it may affect. This is the first page of Cancer.Net’s Guide to Pleuropulmonary Blastoma. To see other pages, use the menu on the side of your screen. Think of that menu as a roadmap to this full guide.

Pleuropulmonary blastoma (PPB) is a rare type of childhood lung tumor that begins in the chest either in the lung tissue (pulmonary) or in the tissue that covers the lungs and the inside of the pleura, also called the chest cavity. A tumor begins when normal cells change and grow uncontrollably, forming a mass called a tumor. A tumor can be cancerous or benign. A cancerous tumor is malignant, meaning it can spread to other parts of the body. A benign tumor means the tumor will not spread.

There are four types of PPB:

  • Type I is made up of air pockets called cysts with early evidence that it is cancerous. However, it is difficult to find and diagnose in this early form. It occurs in very young children, who on average are 10 months old, and has a better chance of being successfully treated than Types II and III, described below.
  • Type Ir is similar to Type I, but it does not have cancerous cells. The “r” stands for regressing.
  • Types II and III generally occur in children ages three to four. Types II and III are cancerous tumors and require intensive chemotherapy.

Many patients with PPB have a mutation of the DICER1 RNase-IIIb gene. More details on the different types of PPB are explained in other sections, including more information about genetic factors in Risk Factors.

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If you would like additional introductory information, explore this related item. Please note this link takes you to another section on Cancer.Net:

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Pleuropulmonary Blastoma - Childhood - Statistics

This section has been reviewed and approved by the Cancer.Net Editorial Board, 11/2014

ON THIS PAGE: You will find information about how many people learn they have this type of tumor each year and some general survival information. Remember, survival rates depend on several factors. To see other pages, use the menu on the side of your screen.

PPB is rare. PPB occurs most often in children younger than four. Most kids are diagnosed between the ages of 3 to 4, but some are diagnosed between ages four to eight. PPB is rarely diagnosed in older children, teenagers, and adults. PPB occurs about equally in boys and girls.

The overall survival rate is the percentage of people who survive after the tumor is detected, excluding those who die from other diseases. The overall survival rate of people with PPB is hard to estimate, given the rare nature of this disease. Talk with your doctor about your child’s prognosis and what individual factors it is based on.

Survival statistics should be interpreted with caution. It is not possible to tell a person how long he or she will live with PPB. Survival statistics may not represent advances made in the treatment or diagnosis of PPB. Learn more about understanding statistics.

Source: International PPB Registry

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Pleuropulmonary Blastoma - Childhood - Risk Factors

This section has been reviewed and approved by the Cancer.Net Editorial Board, 11/2014

ON THIS PAGE: You will find out more about the factors that increase the chance of developing this type of tumor. To see other pages, use the menu on the side of your screen.

A risk factor is anything that increases a person’s chance of developing a tumor. Although risk factors often influence the development of a tumor, most do not directly cause a tumor. Some people with several risk factors never develop a tumor, while others with no known risk factors do.

The cause of PPB is not known. Most often it develops randomly. For most children with PPB, there may have been no personal or family history of the disease before the PPB occurred; in other words, there was no reason to suspect that the child would develop PPB. Most family members of children with PPB are healthy. The following factors may raise a person’s risk of developing PPB.

Cysts. Early stages of PPB appear as cysts. However, most children with lung cysts do not have PPB.

Family history/genetics. Many patients with PPB have a mutation of the DICER1 RNase-IIIb gene. The DICER1 syndrome is also known as Pleuropulmonary Blastoma Family Tumor and Dysplasia syndrome. PPB does not necessarily occur in all families with this gene mutation. This gene mutation is also associated with other cancers such as cystic nephroma, cervical rhabdomyosarcoma, nasal chondromesenchymal hamartoma, ovarian stromal tumors nodular thyroid hyperplasia, ciliary body medulloepithelioma and pineoblastoma.

To continue reading this guide, use the menu on the side of your screen to select another section.

Pleuropulmonary Blastoma - Childhood - Symptoms and Signs

This section has been reviewed and approved by the Cancer.Net Editorial Board, 11/2014

ON THIS PAGE: You will find out more about body changes and other things that can signal a problem that may need medical care. To see other pages, use the menu on the side of your screen.

Children with PPB may experience the following symptoms or signs. Sometimes, children with PPB do not show any of these symptoms. Or, these symptoms may be caused by a medical condition that is not a tumor. Because PPB is so rare, doctors often believe at first that the symptoms are caused by a common childhood illness.

There are two common sets of symptoms that may indicate PPB:

  • Sudden, stressful breathing may be caused by air escaping from the lung cysts into the chest cavity. This is called pneumothorax, meaning there is air in the chest cavity. However, there are many other causes of pneumothorax.
  • Symptoms of PPB may be exactly the same as symptoms of pneumonia, a lung/respiratory infection. These symptoms may include generally feeling unwell, cough, fever, and pain in the chest. Even when a chest x-ray is done, it may be first interpreted as pneumonia. It is common for children with PPB to have been treated for two to three weeks for pneumonia before more tests show that they do not have an infection, but likely have a tumor in the chest.

In addition, sometimes a lung cyst or tumor may be found when a chest x-ray is taken for another reason. See the Diagnosis section for more information on chest x-rays.

If you are concerned about one or more of the symptoms or signs on this list, please talk with your child’s doctor. Your doctor will ask how long and how often your child has been experiencing the symptom(s), in addition to other questions. This is to help find out the cause of the problem, called a diagnosis.

If a tumor is diagnosed, relieving symptoms remains an important part of your child’s medical care and treatment. This may also be called symptom management, palliative care, or supportive care. Be sure to talk with the health care team about symptoms your child experiences, including any new symptoms or a change in symptoms.

The next section helps explain what tests and scans may be needed to learn more about the cause of the symptoms. Use the menu on the side of your screen to select Diagnosis, or you can select another section, to continue reading this guide.  

Pleuropulmonary Blastoma - Childhood - Diagnosis

This section has been reviewed and approved by the Cancer.Net Editorial Board, 11/2014

ON THIS PAGE: You will find a list of the common tests, procedures, and scans that doctors can use to find out what’s wrong and identify the cause of the problem. To see other pages, use the menu on the side of your screen. 

Doctors use many tests to diagnose a tumor and find out whether it has spread to another part of the body, called metastasis. Some tests may also determine which treatments may be the most effective. For most types of tumors, a biopsy is the only way to make a definitive diagnosis. If a biopsy is not possible, the doctor may suggest other tests that will help make a diagnosis. Imaging tests may be used to find out whether the tumor has spread. This list describes options for diagnosing this type of cancer, and not all tests listed will be used for every person.

The diagnosis of PPB depends on an examination under a microscope of material from inside the chest, either cyst material or solid tumor tissue. In Type I, or cystic, PPB, the cysts appear only slightly abnormal, but very close evaluation shows that the walls of the cysts contain very small collections of cancerous cells. In Types II and III PPB, it is obvious when looking under a microscope that tissue inside the chest is cancerous, but because PPB is so rare, it may be difficult for the doctors to determine exactly what type of tumor it is. In Types II and III PPB, it can spread to the heart so doctors might also check the great vessels of the heart and the chest cavity as well. Often, doctors will send tissue samples to other experts for help determining the correct diagnosis.

In addition to a physical examination, the following tests may be used to diagnose PPB:

Biopsy. A biopsy is the removal of a small amount of tissue for examination under a microscope. Other tests can suggest that a tumor is present, but only an examination of a piece of the tumor can make a definite diagnosis. The sample removed during the biopsy is analyzed by a pathologist. A pathologist is a doctor who specializes in interpreting laboratory tests and evaluates cells, tissues, and organs to diagnose disease.

X-ray. An x-ray is a way to create a picture of the structures inside of the body using a small amount of radiation.

Computed tomography (CT or CAT) scan. A CT scan creates a three-dimensional picture of the inside of the body with an x-ray machine. A computer then combines these images into a detailed, cross-sectional view that shows any abnormalities or tumors. A CT scan can also be used to measure the tumor’s size. Sometimes, a special dye called a contrast medium is given before the scan to provide better detail on the image. This dye can be injected into a patient’s vein or given as a pill to swallow. After a chest x-ray shows something abnormal in the lungs, a CT scan is the best method for obtaining more information.

Magnetic resonance imaging (MRI). An MRI uses magnetic fields, not x-rays, to produce detailed images of the body. MRI can also be used to measure the tumor’s size. A special dye called a contrast medium is given before the scan to create a clearer picture. This dye can be injected into a patient’s vein or given as a pill to swallow. An MRI is not a very good way to look inside the chest, but it is often the best test for looking at other parts of the body to determine if the tumor has spread.

Bone scan. A bone scan uses a radioactive tracer to look at the inside of the bones. The tracer is injected into a patient’s vein. It collects in areas of the bone and is detected by a special camera. Healthy bone appears gray to the camera, and areas of injury, such as those caused by a tumor, appear dark.

After diagnostic tests are done, your child’s doctor will review all of the results with you. If the diagnosis is PPB, these results also help the doctor describe the tumor; this is called staging.

The next section helps explain the different stages for this type of tumor. Use the menu on the side of your screen to select Stages, or you can select another section, to continue reading this guide.  

Pleuropulmonary Blastoma - Childhood - Stages

This section has been reviewed and approved by the Cancer.Net Editorial Board, 11/2014

ON THIS PAGE: You will learn about how doctors describe a tumor’s growth or spread. This is called the stage. To see other pages, use the menu on the side of your screen.

Staging is a way of describing where a tumor is located, if or where it has spread, and whether it is affecting other parts of the body. Doctors use diagnostic tests to find out the tumor's stage, so staging may not be complete until all of the tests are finished. Knowing the stage helps the doctor to decide what kind of treatment is best and can help predict a patient's prognosis, which is the chance of recovery. There are different stage descriptions for different types of tumors.

The following criteria help doctors describe PPB:

Type I: Type I PPB generally occurs in children younger than one year and is made up of mostly cysts, with almost no small, solid tumors that are called nodules. A thin layer of the wall of the cyst may appear cancerous. Type I PPB usually has the best prognosis of the different types.

Type Ir: Type Ir is made up of cysts that do not contain cancerous cells.

Type II: Type II PPB contains both cysts and cancerous nodules, and occurs most commonly in children around three years old.

Type III: Type III PPB is a solid cancerous tumor, and occurs most often in children around four years old.

For Types II and III, a bone scan and MRI (see Diagnosis) may be necessary to detect the stage.

Recurrent: Recurrent cancer is cancer has come back after treatment. If there is a recurrence, the tumor may need to be staged again using the criteria above in a process called restaging.

Sources: Dehner LP: Pleuropulmonary blastoma is THE pulmonary blastoma of childhood. Seminars in Diagnostic Pathology 11:144-51, 1994, and The International PPB Registry.

Information about PPB’s stage will help the doctor recommend a treatment plan.  The next section helps explain the treatment options for this type of cancer. Use the menu on the side of your screen to select Treatment Options, or you can select another section, to continue reading this guide.  

Pleuropulmonary Blastoma - Childhood - Treatment Options

This section has been reviewed and approved by the Cancer.Net Editorial Board, 11/2014

ON THIS PAGE: You will learn about the different ways doctors use to treat children with this type of tumor. To see other pages, use the menu on the side of your screen.

In general, tumors in children are uncommon, so it can be hard for doctors to plan treatments unless they know what has been most effective in other children. That’s why more than 60% of children with cancer are treated as part of a clinical trial. Clinical trials are research studies that compare standard treatments, which are the best proven treatments available, with newer approaches to treatments that may be more effective. Clinical trials may test such approaches as a new drug, a new combination of standard treatments, or new doses of current therapies. Studying new treatments involves careful monitoring using scientific methods, and all participants are followed closely to track their health and progress.

To take advantage of these newer treatments, all 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. 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, nutritionists, physical and occupational therapists, social workers, and counselors. Special activities and programs to help your child and family cope may also be available.

Descriptions of treatment options for PPB are listed 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 may also include treatment for symptoms and side effects, an important part of cancer care. Surgery and chemotherapy, and sometimes radiation therapy, are used to treat PPB. If all of the tumor cannot be removed during surgery, chemotherapy and/or radiation therapy may be used to kill the remaining cancerous cells.

Take time to learn about all of your child’s treatment options and be sure to ask questions about things that are unclear. Also, talk about the goals of each treatment with your child’s doctor and what your child can expect while receiving the treatment. Learn more about making treatment decisions.

Surgery

Surgery is the removal of the tumor and surrounding 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 performed before surgery to reduce the size of the tumor. This is called pre-operative chemotherapy.

Side effects from surgery for PPB can vary. Talk with your child’s doctor about possible short-term and long-term side effects. Learn more about cancer surgery.

Chemotherapy

Chemotherapy is the use of drugs to destroy tumor cells, usually by stopping the tumor cells’ ability to grow and divide. Chemotherapy is given by a medical oncologist, a doctor who specializes in treating cancer with medication.

Systemic chemotherapy is delivered through the bloodstream to reach cancer cells throughout the body. Common ways to give chemotherapy include an intravenous (IV) tube placed into a vein using a needle or in a pill or capsule that is swallowed.

A chemotherapy regimen, which is your child’s treatment plan, usually consists of a specific number of cycles given over a set period of time. A patient may receive one drug at a time or combinations of different drugs 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.

Chemotherapy may benefit patients with Type I PPB to reduce the risk of recurrence. 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 to kill any remaining PPB cells; this is called adjuvant or post-operative chemotherapy. Sometimes, pre-operative chemotherapy is given.

Common drugs for Type 1 PPB include: cyclophosphamide (Neosar), vincristine (Vincasar) and dactinomycin (Cosmegen). Specific drugs for Types II and III often includes 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 once treatment is finished.

Learn more about chemotherapy and preparing for treatment. The medications used to treat PPB are continually being evaluated. Talking with your child’s doctor is often the best way to learn about the medications prescribed for your child, their purpose, and their potential side effects or interactions with other medications. Learn more about your prescriptions by using searchable drug databases.

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 usually consists of a specific number of treatments given over a set period of time.

Not every child with PPB receives radiation therapy. The decision to use radiation therapy is made if 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 radiation therapy.

Getting care for symptoms and side effects

PPB and its treatment often cause side effects. In addition to treatment to slow, stop, or eliminate the tumor, an important part of care is relieving a person’s symptoms and side effects. This approach is called palliative or supportive care, and it includes supporting the patient with his or her physical, emotional, and social needs.

Palliative care can help a person at any stage of illness. People often receive treatment for the tumor and treatment to ease side effects at the same time. In fact, patients who receive both often have less severe symptoms, better quality of life, and report they are more satisfied with treatment.

Palliative treatments vary widely and often include medication, nutritional changes, relaxation techniques, and other therapies. Your child may also receive palliative treatments similar to those meant to eliminate the tumor, such as chemotherapy, surgery, and radiation therapy. Talk with your child’s doctor about the goals of each treatment in the treatment plan.

Before treatment begins, talk with your child’s health care team about the possible side effects of the specific treatment plan and supportive care options. And during and after treatment, be sure to tell your child’s doctor or another health care team member if your child is experiencing a problem so it can be addressed as quickly as possible. Learn more about palliative care.

Remission and the chance of recurrence

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

A remission can be temporary or permanent. This uncertainty leads many patients and families to feel worried or anxious that the tumor 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 the 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 does return after the original treatment, it is called recurrent tumor. It may come back in the same place, meaning it is a local recurrence, or nearby, which is a regional recurrence. If it comes back in another place, it is a distant recurrence.

When this occurs, a 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 therapies 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.

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.

When a tumor recurs, patients and their families often experience emotions such as disbelief or fear. Families are encouraged to talk with their health care team about these feelings and ask about support services to help them cope. Learn more about dealing with recurrence.

If treatment fails

Although treatment is successful for the majority of children with cancer, sometimes it is not. If a child’s tumor cannot be cured or controlled, this is called advanced or terminal disease. This diagnosis is stressful, and it may be difficult to discuss. However, it is important to have open and honest conversations with your child’s doctor and health care team to express your family’s feelings, preferences, and concerns. The health care team is there to help, and many team members have special skills, experience, and knowledge to support patients and their families.

Advanced PPB is when the disease 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. PPB that has spread is rarely treated with surgery; other treatments will be recommended. If one 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.

Parents or guardians are encouraged to think about where the child would be most comfortable: at home, in a home-like setting elsewhere, in the hospital, or in a hospice environment. Hospice care is a type of palliative care for people who are expected to live less than six months. It is designed to provide the best possible quality of life for people who are near the end of life. Nursing care and special equipment can make staying at home a workable alternative for many families. Some children may be happier if they can arrange to 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 helps explain clinical trials, which are research studies. Use the menu on the side of your screen to select About Clinical Trials, or you can select another section, to continue reading this guide.  

Pleuropulmonary Blastoma - Childhood - About Clinical Trials

This section has been reviewed and approved by the Cancer.Net Editorial Board, 11/2014

ON THIS PAGE: You will learn more about clinical trials, which are the main way that new medical approaches are tested to see how well they work. To see other pages, use the menu on the side of your screen.

Doctors and scientists are always looking for better ways to treat children with PPB. To make scientific advances, doctors create research studies involving volunteers, called clinical trials.

Many clinical trials are focused on new treatments, evaluating whether a new treatment is safe, effective, and possibly better than the standard treatment. These types of studies evaluate new drugs, different combinations of existing treatments, new approaches to radiation therapy or surgery, and new methods of treatment. Children who participate in clinical trials are often among the first to receive new treatments before they are widely available. However, there is no guarantee that the new treatment will be safe, effective, or better than a standard treatment.

There are also clinical trials that study new ways to ease symptoms and side effects during treatment and manage the late effects that may occur after treatment. Talk with your child’s doctor about clinical trials regarding side effects. In addition, there are ongoing studies about ways to prevent the disease.

Patients decide to participate in clinical trials for many reasons. For some patients, a clinical trial is the best treatment option available. Because standard treatments are not perfect, patients are often willing to face the added uncertainty of a clinical trial in the hope of a better result. Other patients volunteer for clinical trials because they know that these studies are the only way to make progress in treating PPB. Even if they do not benefit directly from the clinical trial, their participation may benefit future children with PPB.

Sometimes people have concerns that, by participating in a clinical trial, they may receive no treatment by being given a placebo or a “sugar pill.” The use of placebos in cancer clinical trials is rare. When a placebo is used in a study, it is done with the full knowledge of the participants. Find out more about placebos in cancer clinical trials.

To join a clinical trial, parents and children must participate in a process known as informed consent. During informed consent, the doctor should list all of the patient’s options, so that the person understands how the new treatment differs from the standard treatment. The doctor must also list all of the risks of the new treatment, which may or may not be different from the risks of standard treatment. Finally, the doctor must explain what will be required of each patient in order to participate in the clinical trial, including the number of doctor visits, tests, and the schedule of treatment.

For specific topics being studied for PPB, learn more in the Latest Research section.

Patients who participate in a clinical trial may stop participating at any time for any personal or medical reason. This may include that the new treatment is not working or there are serious side effects. Clinical trials are also closely monitored by experts who watch for any problems with each study. It is important that parents and children participating in a clinical trial talk with their doctor and researchers about who will be providing their treatment and care during the clinical trial, after the clinical trial ends, and/or if the patient chooses to leave the clinical trial before it ends. 

Cancer.Net offers a lot of information about clinical trials in other areas of the website, including a complete section on clinical trials and places to search for clinical trials for a specific type of cancer.

The next section helps explain the areas of research going on today about this type of cancer. Use the menu on the side of your screen to select Latest Research, or you can select another section, to continue reading this guide.  

Pleuropulmonary Blastoma - Childhood - Latest Research

This section has been reviewed and approved by the Cancer.Net Editorial Board, 11/2014

ON THIS PAGE: You will read about the scientific research being done now to learn more about this type of tumor and how to treat it. To see other pages, use the menu on the side of your screen.

Doctors are working to learn more about PPB, ways to prevent it, how to best treat it, and how to provide the best care to people diagnosed with this disease. The following areas of research may include new options for patients through clinical trials. Always talk with your child’s doctor about the diagnostic and treatment options best for your child.

Drug combinations. The experimental drug gefitinib (Iressa) is being tested in combination with irinotecan (Camptosar) and vincristine (Vincasar) to treat PPB in children. Cefixime (Suprax) and cefpodoxime (Vantin) are also added to the combination to reduce severe diarrhea for some patients.

Genetic causes of PPB. As explained in Risk Factors, researchers are continuing to investigate the link between a genetic mutation to DICER1 and familial PPB.

Research into causes and treatment for a rare tumor like PPB requires collecting information from many hospitals. The International PPB Registry is the largest such collection of information on PPB in the world. The Registry has approval from the participating institutions’ Institutional Review Boards to ensure the protection of patients’ privacy.

Treatment guidelines. Currently, there are no large-scale organized treatment schedules for PPB due to it being so rare. Individual doctors use their experience in treating similar conditions, such as soft-tissue sarcomas, to guide their PPB treatment recommendations. Plans are underway to create an international consortium of pediatric oncology specialists from around the world to consider treatment options and make recommendations for treating people with PPB.

Supportive care. Clinical trials are underway to find better ways of reducing symptoms and side effects of current PPB treatments in order to improve patients’ comfort and quality of life.

Looking for More about Latest Research?

If you would like additional information about the latest areas of research regarding PPB, explore these related items that take you outside of this guide:

  • To find clinical trials specific to your diagnosis, talk with your child’s doctor or search online clinical trial databases now.
  • Visit ASCO’s CancerProgress.Net website to learn more about the historical pace of research for childhood cancer. Please note this link takes you to a separate ASCO website. 

The next section addresses how to cope with the symptoms of the disease or the side effects of its treatment. Use the menu on the side of your screen to select Coping with Side Effects, or you can select another section, to continue reading this guide.  

Pleuropulmonary Blastoma - Childhood - Coping with Side Effects

This section has been reviewed and approved by the Cancer.Net Editorial Board, 11/2014

ON THIS PAGE: You will find out more about steps to take to help cope with physical, social, and emotional side effects. This page includes several links outside of this guide to other sections of this website. To see other pages, use the menus on the side of your screen.

Fear of treatment side effects is common after a diagnosis of cancer, but it may help to know that preventing and controlling side effects is a major focus of your child’s health care team. This is called palliative or supportive care, and it is an important part of the overall treatment plan, regardless of the stage of disease.

Common side effects from each treatment option for PPB are described in detail within the Treatment Options section. Learn more about the most common side effects of a tumor and different treatments, along with ways to prevent or control them. Side effects depend on a variety of factors, including the type of PPB, the length and dosage of treatment(s), and your child’s overall health.

Before treatment begins, talk with your child’s doctor about possible side effects of each type of treatment your child will be receiving. Ask which side effects are most likely to happen, when they are likely to occur, and what can be done to prevent or relieve them. And, ask about the level of caregiving your child may need during treatment and recovery, as family members and friends often play an important role in the care of a person with PPB. Learn more about caregiving.

In addition to physical side effects, there may be emotional and social effects as well. Children and their families are encouraged to share their feelings with a member of their health care team who can help with coping strategies, including concerns about managing the cost of your child's medical care

During and after treatment, be sure to tell the health care team about the side effects your child experiences, even if you feel they are not serious. Sometimes, side effects can last beyond the treatment period, called a long-term side effect. A side effect that occurs months or years after treatment is called a late effect. Treatment of both types of effects is an important part of survivorship care. Learn more by reading the After Treatment section or talking with your child’s doctor.

 The next section helps explain medical tests and check-ups needed after finishing treatment. Use the menu on the side of your screen to select After Treatment, or you can select another section, to continue reading this guide.  

Pleuropulmonary Blastoma - Childhood - After Treatment

This section has been reviewed and approved by the Cancer.Net Editorial Board, 11/2014

ON THIS PAGE: You will read about your child’s medical care after treatment is finished and why this follow-up care is important. To see other pages, use the menu on the side of your screen.  

After treatment for PPB ends, talk with your child’s doctor about developing a follow-up care plan. This plan may include regular physical examinations and/or medical tests to monitor your child’s recovery for the coming months and years. All children treated for a tumor, including PPB, should have life-long, follow-up care.

If a recurrence happens, it is more likely to occur in the first few years after the original treatment. Therefore, regular screening should continue for two to three years, or possibly up to five years, after diagnosis. Very rarely, PPB returns after these standard periods of follow-up. It is important to note that PPB can rapidly grow, and this possibility should be discussed with your child’s doctor, including what signs and symptoms to watch for.

Many of the current treatments that are effective in treating childhood tumors are associated with side effects, both in the short and long term. Life-long follow-up care is based on the dosage and specific chemotherapy used in treatment, as well as the dosage of radiation therapy. Recommendations for follow-up care according to the risk of possible late effects of treatment have been developed by the Children's Oncology Group and can be found at CureSearch.org.

Based on the type of treatment your child received, the doctor will determine what examinations and tests are needed to check for long-term side effects and the possibility of secondary cancers. Your child’s doctor can recommend the necessary screening tests. Follow-up care should also address your child’s quality of life, including any developmental or emotional concerns. Learn more about childhood cancer survivorship.

The child’s family is encouraged to organize and keep a record of the child’s medical information. That way, as the child enters adulthood, he or she has a clear, written history of the diagnosis, the treatment given, and the doctor’s recommendations about the schedule for follow-up care. The doctor’s office can help you create this. This information will be valuable to doctors who care for your child during his or her lifetime. ASCO offers cancer treatment summary forms to help keep track of the treatment your child received and develop a survivorship care plan once treatment is completed.

Children who have had PPB can also enhance the quality of their future by following established guidelines for good health into and through adulthood, including not smoking, maintaining a healthy weight, eating a balanced diet, and participating in regular physical activity. Talk with your child’s doctor about developing a plan that is best for your child’s needs. Learn more about the next steps to take in survivorship.

The next section offers a list of questions you may want to ask. Use the menu on the side of your screen to select Questions to Ask the Doctor, or you can select another section, to continue reading this guide.  

Pleuropulmonary Blastoma - Childhood - Questions to Ask the Doctor

This section has been reviewed and approved by the Cancer.Net Editorial Board, 11/2014

ON THIS PAGE: You will find some questions to ask your doctor or other members of your health care team, to help you better understand your child’s diagnosis, treatment plan, and overall care. To see other pages, use the menu on the side of your screen.

Talking often with the doctor is important to make informed decisions about your child’s health care. These suggested questions are a starting point to help you learn more about your child’s care and treatment. You are also encouraged to ask additional questions that are important to you. You may want to print this list and bring it to your child’s next appointment, or download Cancer.Net’s free mobile app for an e-list and other interactive tools to manage your child’s care.

  • What type of PPB has been diagnosed? What does this mean?
  • Can you explain my child’s pathology report, or laboratory test results, to me?
  • What is your familiarity with PPB and its treatment?
  • Do you collaborate with the International PPB Registry?
  • What are my child’s treatment options?
  • What is the goal of each treatment? Is it to eliminate the cancer, help my child feel better, or both?
  • What clinical trials are open to my child? Where are they located, and how do I find out more about them?
  • What treatment plan do you recommend? Why?
  • What is my child’s prognosis?
  • Should I seek a second opinion?
  • Does your practice include multidisciplinary care?
  • Who will be part of my child’s health care team, and what does each member do?
  • Who will be coordinating my child’s overall treatment and follow-up care?
  • Do you attend expert meetings to discuss complicated tumor cases? What kinds of specialists attend such meetings?
  • Will my child have surgery? If so, can you describe the surgery and recovery to me?
  • Will my child receive chemotherapy? What chemotherapy will be given? How long and how frequently?
  • Will my child receive radiation therapy?
  • What are the possible side effects of each treatment, both in the short term and the long term?
  • How will this treatment affect my child’s daily life? Will he or she be able to attend school and perform his or her usual activities?
  • Is there anything we can do before starting treatment to avoid late effects?
  • Could this treatment affect my child’s ability to become pregnant or have children in the future? If so, should I talk with a fertility specialist before cancer treatment begins?
  • How long will my child stay in the hospital?
  • If I am worried about managing the costs related to my child’s care, who can help me with these concerns?
  • What follow-up tests will my child need, and how often will he or she need them?
  • What support services are available to my child? To my family?
  • Whom should I call for questions or problems?

The next section offers some more resources that may be helpful to you. Use the menu on the side of your screen to select Additional Resources, or you can select another section, to continue reading this guide.  

Pleuropulmonary Blastoma - Childhood - Additional Resources

This section has been reviewed and approved by the Cancer.Net Editorial Board, 11/2014

ON THIS PAGE: You will find some helpful links to other areas of Cancer.Net that provide information about cancer care and treatment. This is the final page of Cancer.Net’s Guide to Pleuropulmonary Blastoma. To go back and review other pages, use the menu on the side of your screen.

Cancer.Net includes many other sections about the medical and emotional aspects of cancer, both for the person diagnosed and their family members and friends. This website is meant to be a resource for you and your loved ones from the time of diagnosis, through treatment, and beyond. Here are a few sections that may get you started in exploring the rest of Cancer.Net:

Search for a cancer specialist in your local area using this free database of doctors from the American Society of Clinical Oncology.

Review dictionary articles to help understand medical phrases and terms used in cancer care and treatment.

- Read more about the first steps to take when your child is newly diagnosed with a tumor.

- Find out more about clinical trials as a treatment option.

Learn more about coping with the emotions that a tumor can bring, including those within a family or a relationship.

Find a national, not-for-profit advocacy organization that may offer additional information, services, and support for people with this type of tumor.

- Explore next steps a child can take after active treatment is complete.

This is the end of Cancer.Net’s Guide to Pleuropulmonary Blastoma. Use the menu on the side of your screen to select another section to continue reading this guide.