1. A Very Rare Disease
Pleuropulmonary Blastoma, or PPB, is a rare lung cancer found in children. This is one of the only cancers to begin in the lungs of a child. PPB forms in the lung tissue, the outer covering of the lungs or on the inner lining of the chest cavity. This rare type of cancer affects children from birth to 12 years old. It is most common in three to four year olds. In a very few rare instances, PPB has been found in teens. PPB gets its name from its position on the lungs and the fact that it is a very primitive form of cancer. Only 20 to 30 cases of PPB are diagnosed worldwide each year.
2. Common Symptoms
This is a childhood cancer rarely considered by any physician because the symptoms of the disease are normal in most children. These symptoms include cough, fever and pain in the chest. Most physicians will consider things such as a respiratory infection when a child presents with these symptoms. Sometimes, a doctor may misdiagnose this as pneumonia because the two diseases look similar on x-rays. Because this is such a rare disease, some physicians have never heard of it.
3. Limited Options
Treating PPB is difficult because it is such a rare childhood cancer. Until the 1980's, there was very little research at all for doctors treating PPB patients to consider. Surgery, chemotherapy and radiation therapy are all considered good ways to treat PPB. Surgery is the best option when possible. There's not yet any documentation on immunotherapy for PPB. When trying to come up with a treatment plan, doctors will consult with the child's parents, a social worker and often other doctors from around the world. If doctors detect PPB early and if treatment begins immediately, the survival rate can be as high as 80 percent.
4. Cancer on the Move
When a child receives a diagnosis of PPB, immediate treatment is necessary because this cancer can spread to distant organs, such as the liver or brain, through the blood stream. PPB can travel to bones as well. It is imperative that the doctor treating the child order a full battery of tests, including a CT scan to check the entire for signs of the cancer. If the cancer spreads, treatment is more difficult. This treatment will usually include more chemotherapy and radiation therapy.
5. Support is Necessary
Finding out that your child has PPB is difficult. Because this type of cancer is so rare, finding support may prove to be a challenge. Ask your doctor about the International Pleuropulmonary Blastoma Registry or the Rare Cancer Alliance. Both of these organizations can help you to contact people who can help to support you.
