This study is about treating a rare lung tumor in kids called pleuropulmonary blastoma (PPB). There are three types of PPB: Type I, II, and III. The study is testing different ways to treat these tumors. For Type I, they are comparing surgery with and without chemotherapy (a drug treatment that kills cancer cells). For Types II and III, they are adding a new medicine, called topotecan, to the usual chemotherapy to see if it works better.

PPB treatment can include medicines like vincristine, topotecan, cyclophosphamide, and others. These medicines work by stopping tumor cells from growing or by killing them. The study will look at how well these treatments work and any side effects they may cause.

• Study Length: The study includes several cycles of treatment, with each cycle lasting 21 days.
• Visits Needed: Regular visits for treatment and check-ups over several months are required.
• Eligibility: Kids age 21 or younger with newly diagnosed PPB can join. Pregnant girls cannot participate.