Pleuropulmonary Blastoma - Childhood

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 its rarity. 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 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. 

Learn more about common statistical terms used in medical research.

Looking for More about Current Research? 

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

• To find clinical trials specific to your diagnosis, talk with your child’s doctor or search online clinical trial databases now.
• Read ASCO’s latest Clinical Cancer Advances report (PDF), which highlights top research findings over the past year.

• Visit ASCO’s CancerProgress.Net website to learn more about the historical pace of research for childhood cancer.

Or, choose “Next” (below, right) to continue reading this detailed section.