Pleuropulmonary Blastoma – Childhood

Overview

Pleuropulmonary blastoma (PPB) is a rare type of childhood lung tumor that begins in the chest either in the lung tissue itself (pulmonary) or in the tissue that covers the lungs and the inside of the chest cavity (pleura). A tumor begins when normal cells begin to change and grow uncontrollably. A tumor can be benign (noncancerous) or malignant (cancerous, meaning it can spread to other parts of the body).
There are four types of PPB:

• Type I is made up of cysts (air pockets) with early evidence that it is cancerous, but it is difficult to diagnose and may not be found in this early form. It occurs in very young children and has a better chance of being successfully treated than Types II and III (see below).
• Type Ir (the “r” stands for regressing) is similar to Type I, but it does not have cancerous cells.
• Types II and III generally occur in children ages two to four. Types II and III are cancerous tumors and require intensive chemotherapy.

Symptoms

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 a tumor. 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.

Surgery

Surgery is the main treatment for PPB. A surgical oncologist is a doctor who specializes in treating cancer using surgery. The goal of surgery is to completely remove the tumor. If the tumor is too large to be completely removed, or if it is not possible to completely remove the tumor, surgery may be performed after chemotherapy (called pre-operative chemotherapy).
Side effects from surgery for PPB can vary, and patients are encouraged to discuss possible short-term and long-term side effects with their doctor.

Chemotherapy

Chemotherapy is the use of drugs to kill cancer cells. Systemic chemotherapy is delivered through the bloodstream, targeting cancer cells throughout the body. Chemotherapy is given by a medical oncologist, a doctor who specializes in treating cancer with medication. Some people may receive chemotherapy in their doctor’s office; others may go to the hospital. A chemotherapy regimen (schedule) usually consists of a specific number of cycles given over a specific time. There is much experience using many drugs for treating childhood cancer. Even children as young as newborn infants can be safely treated for life-threatening cancers with chemotherapy.

Radiation therapy

Radiation therapy is the use of high-energy x-rays or other particles to kill cancer cells. A doctor who specializes in giving radiation therapy to treat cancer 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.

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