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 usually occurs in infants and young children. Learn more about the risk factors, symptoms, diagnosis, and treatment of pleuropulmonary blastoma.
Pleuropulmonary blastoma
Image by Terese Winslow (Illustrator) / NCI
What Is
Baby Sitting Showing Respiratory System
Image by TheVisualMD
Baby Sitting Showing Respiratory System
A baby's first breath is dramatic for all kinds of reasons, including physiological ones. A protein-lipid combination called surfactant plays a critical role in lung development. Researchers have discovered that nutrients such as vitamin D also play important roles in the healthy development of the infant's respiratory system. One of the most obvious ways that we notice the overall immune health of a baby is through the presence or absence of respiratory infections. Respiratory illness is the leading cause of hospitalization among young children. And when it is severe enough to require hospitalization, respiratory illness greatly increases the risk of childhood asthma.
Image by TheVisualMD
What Is Pleuropulmonary Blastoma?
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 DICER1 gene.
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.
Source: Childhood Pleuropulmonary Blastoma Treatment (PDQ®)–Patient Version - National Cancer Institute
Additional Materials (1)
AACR 2009: Genetics of pleuropulmonary blastoma
Video by ecancer/YouTube
10:20
AACR 2009: Genetics of pleuropulmonary blastoma
ecancer/YouTube
Types
What Are the Types of Pleuropulmonary Blastoma?
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.
Source: Childhood Pleuropulmonary Blastoma Treatment (PDQ®)–Patient Version - National Cancer Institute
Risk Factors
Mouse dicer
Image by www.rcsb.org/Wikimedia
Mouse dicer
Structure of RNaseIIIb and dsRNA binding domains of mouse Dicer as provided by RCSB.
Image by www.rcsb.org/Wikimedia
What Increases the Risk of Pleuropulmonary Blastoma?
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.
Source: Childhood Pleuropulmonary Blastoma Treatment (PDQ®)–Patient Version - National Cancer Institute
Additional Materials (1)
Locations of germline mutations known for DICER1 syndrome.
Locations of germline mutations known for DICER1 syndrome.
Image by Robertson, J.C.; Jorcyk, C.L.; Oxford, J.T. DICER1 Syndrome: DICER1 Mutations in Rare Cancers. Cancers 2018, 10, 143./Wikimedia
Locations of germline mutations known for DICER1 syndrome.
Image by Office of Biological and Environmental Research of the U.S. Department of Energy Office of Science, the Biological and Environmental Research Information System, Oak Ridge National Laboratory.
Ideogram of human chromosome 14
Selected genes, traits, and disorders associated with the chromosome listed; (blue and violet) regions reflecting the unique patterns of light and dark bands seen on human chromosomes stained to allow viewing through a light microscope; (red) the centromere, or constricted portion, of each chromosome; (yellow) chromosomal regions that vary in staining intensity and sometimes are called hererochromatin (meaning “different color”); (lines between yellow) variable regions, called stalks, that connect a very small chromosome arm (a “satellite”) to the chromosome.
Image by Office of Biological and Environmental Research of the U.S. Department of Energy Office of Science, the Biological and Environmental Research Information System, Oak Ridge National Laboratory.
DICER1 Gene: Dicer 1, Ribonuclease III
Normal Function
The DICER1 gene provides instructions for making a protein that plays a role in regulating the activity (expression) of other genes. The Dicer protein aids in the production of a molecule called microRNA (miRNA). MicroRNAs are short lengths of RNA, a chemical cousin of DNA. Dicer cuts (cleaves) precursor RNA molecules to produce miRNA.
MicroRNAs control gene expression by blocking the process of protein production. In the first step of making a protein from a gene, another type of RNA called messenger RNA (mRNA) is formed and acts as the blueprint for protein production. MicroRNAs attach to specific mRNA molecules and stop the process by which protein is made. Sometimes, miRNAs break down the mRNA, which also blocks protein production. Through this role in regulating the expression of genes, Dicer is involved in many processes, including cell growth and division (proliferation) and the maturation of cells to take on specialized functions (differentiation).
Health Conditions Related to Genetic Changes
DICER1 syndrome
Mutations in the DICER1 gene cause DICER1 syndrome. People with this condition have an increased risk of developing many types of tumors, particularly certain tumors of the lungs (pleuropulmonary blastoma); kidneys (cystic nephroma); ovaries (Sertoli-Leydig tumors); and thyroid, a butterfly-shaped gland in the lower neck (multinodular goiter). Most of these mutations lead to an abnormally short Dicer protein that is likely unable to produce miRNA. Without regulation by miRNA, genes are expressed abnormally, which could cause cells to grow and divide uncontrollably and lead to tumor formation.
What Are the Signs and Symptoms of Pleuropulmonary Blastoma?
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.
Source: Childhood Pleuropulmonary Blastoma Treatment (PDQ®)–Patient Version - National Cancer Institute
Diagnosis
Cancer Imaging
Image by TheVisualMD
Cancer Imaging
Medical imaging technology has revolutionized the screening, study and treatment of cancer. Imaging is used before, during, and after cancer treatment: Screening. Generally used for people at increased risk of certain types of cancer, screening can help determine if there are abnormal areas present that may be cancerous.
Diagnosis and staging. Imaging can be used to find out how much cancer is in the body, where it’s located, and if it’s metastasized. Imaging can also be used as an aid in biopsy, to guide the doctors to the tumor.
Treatment. Imaging can help to guide cancer treatments by exactly determining tumor location, so that treatment can focus on the tumor alone to minimize damage to nearby tissue. Imaging can also show if a treatment is working by showing if a tumor is shrinking, has changed, or is using less of the body’s resources.
Monitoring for recurrence. After treatment, imaging can be used to see if a cancer has recurred or if it has metastasized elsewhere.
Image by TheVisualMD
How Is Pleuropulmonary Blastoma Diagnosed?
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.
Source: Childhood Pleuropulmonary Blastoma Treatment (PDQ®)–Patient Version - National Cancer Institute
Stages
Cancer staging
Image by TheVisualMD
Cancer staging
Staging Cancer : Staging is the process of finding out the amount of cancer in the body and if it has spread. Most tumorous cancers are staged using the TNM system. In the TNM system, T = extent of the primary tumor, N = extent of spread to lymph nodes, M = presence of metastasis. After the TNM description has been decided, the cancer can be designated as Stage 0-IV. Stage 0 =carcinoma in situ. In Stage I, Stage II, and Stage III, higher numbers indicate more extensive disease, ie, greater tumor size, and/or spread of the cancer to nearby lymph nodes, and/or organs adjacent to the primary tumor. In Stage IV, the cancer has spread to another organ.
Image by TheVisualMD
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.
Source: Childhood Pleuropulmonary Blastoma Treatment (PDQ®)–Patient Version - National Cancer Institute
Treatment
Treating Cancer
Image by TheVisualMD
Treating Cancer
Because cancers differ from one another in many ways, and because each patient is unique, there isn`t just one approach to treatment. Cancer treatment aims to eliminate the primary tumor, prevent the recurrence or spread of the cancer, and relieve symptoms. Types of cancer treatment include surgery; radiation therapy, which targets specific cancer cells; chemotherapy, which targets cancer cells throughout the body; and biological therapy, which works with the body`s own immune system. If you have cancer, it`s important to educate yourself about the type of cancer you have and the options for its treatment so that you can make informed decisions.
Image by TheVisualMD
How Is Pleuropulmonary Blastoma Treated in Children?
Treatment Option Overview
KEY POINTS
There are different types of treatment for children with pleuropulmonary blastoma.
Children with pleuropulmonary blastoma should have their treatment planned by a team of doctors who are experts in treating childhood cancer.
Two types of standard treatment are used for pleuropulmonary blastoma:
Surgery
Chemotherapy
New types of treatment are being tested in clinical trials.
Targeted therapy
Treatment for pleuropulmonary blastoma may cause side effects.
Patients may want to think about taking part in a clinical trial.
Patients can enter clinical trials before, during, or after starting their cancer treatment.
Follow-up tests may be needed.
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).
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
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.
Treatment of Recurrent Pleuropulmonary Blastoma
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.
Source: Childhood Pleuropulmonary Blastoma Treatment (PDQ®)–Patient Version - National Cancer Institute
Prognosis
Prognosis Icon
Image by mcmurryjulie/Pixabay
Prognosis Icon
Image by mcmurryjulie/Pixabay
What Affects the Prognosis for Pleuropulmonary Blastoma?
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.
Source: Childhood Pleuropulmonary Blastoma Treatment (PDQ®)–Patient Version - National Cancer Institute
Send this HealthJournal to your friends or across your social medias.
Childhood Pleuropulmonary Blastoma
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 usually occurs in infants and young children. Learn more about the risk factors, symptoms, diagnosis, and treatment of pleuropulmonary blastoma.