Pleuropulmonary blastoma


Pleuropulmonary blastoma is a rare form of cancer first reported in 1988. It is commonly found in children. Symptoms may include coughing, an upper respiratory tract infection, shortness of breath, and chest pain.


Children with PPB may experience the following symptoms. Sometimes, children with PPB do not show any of these symptoms. Or, these symptoms may be caused by a medical condition that is not cancer. If you are concerned about a symptom on this list, please talk with your child’s doctor. Because PPB is 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 (air in the chest cavity). However, there are also many other causes of pneumothorax. Symptoms of PPB may be exactly the same as symptoms of pneumonia (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 (see Diagnosis) is taken for another reason.


The cause of PPB is not known. Most often it develops randomly. About 25% of children with PPB have had or have a family history of lung cysts, kidney cysts (called cystic nephroma, which is very rare), thyroid lumps (sometimes cancerous), and other childhood cancers. 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.


Doctors use many tests to diagnose cancer and determine if it has metastasized (spread). Some tests may also determine which treatments may be the most effective. For most types of cancer, a biopsy is the only way to make a definitive diagnosis of cancer. 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 cancer has metastasized. 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 (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 cancer it is. 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 cancer 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 doctor who specializes in interpreting laboratory tests and evaluates cells, tissues, and organs to diagnose disease). X-ray. An x-ray is a picture of the inside of the body. For instance, a chest x-ray can help doctors determine if cancer is in the lungs. 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. Sometimes, a contrast medium (a special dye) is injected into a patient’s vein to provide better detail. 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. A contrast medium may be injected into a patient’s vein to create a clearer picture. 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 cancer 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 cancer, appear dark.


Clinical Trials are the standard of care for the treatment of children with cancer. In fact, more than 60% of children with cancer are treated as part of a clinical trial. Clinical trials are research studies that compare standard treatments (the best treatments available) with newer treatments that may be more effective. Cancer in children is rare, so it can be hard for doctors to plan treatments unless they know what has been most effective in other children. Investigating new treatments involves careful monitoring using scientific methods, and all participants are followed closely to track progress. To take advantage of these newer treatments, all children with cancer should be treated at a specialized cancer center. Doctors at these centers have extensive experience in treating children with cancer and have access to the latest research. Many times, a team of doctors treats a child with cancer. Pediatric cancer centers often have extra support services for children and their families, such as nutritionists, social workers, and counselors. Special activities for kids with cancer may also be available. The treatment of PPB depends on the size and location of the tumor, whether the cancer has spread, and the child’s overall health. 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