In general, surgery is the initial approach to the treatment of solid tumors such as PPB. Surgery allows specific diagnosis of the tumor, as well as alleviation of symptoms caused by the presence of a large amount of abnormal tissue. When PPB presents itself as lung cysts and pneumothorax, removal of the cyst is done. When PPB presents as a large mass in the chest, an attempt is often made to remove the mass. PPBs of this type are often very large and fill half the chest, almost completely compressing lung tissue on one side. Such large tumors often invade many other “structures” in the chest, such as the chest wall and mediastinum (the central area of the chest). It is nearly impossible to remove such large tumors entirely, and it may be dangerous for the surgeon to try to do so. At times, the possibility of removing the tumor can be determined only during a careful surgical appraisal with the decision made by the surgeon at that time. In general it is considered desirable to remove as much of the tumor mass as possible, but statistical analysis in the relatively small number of cases of PPB reported (compared to much more common childhood cancers) has not shown a survival advantage based on the degree of tumor removal. Nevertheless, based on experience with many other cancers, maximum surgical removal is still believed worthwhile and is often attempted.
Neo-adjuvant Chemotherapy (chemotherapy given before attempting major surgery to remove a tumor): If a tumor mass is too large to be removed safely, occasional attempts are made to shrink it with chemotherapy and then try surgery later. This approach is carried out after a biopsy is done for precise diagnosis. This pre-surgery chemotherapy is called "neo-adjuvant chemotherapy". It is recommended in some other childhood cancers, especially when the chemotherapy is very effective and when the tumor is very large to start with. It is a sensible approach to try for very large PPB tumors. There is enough experience now in PPB with several chemotherapy drugs to have modest optimism that large "inoperable" tumors will shrink and be more easily/safely removed with surgery.