Pleuropulmonary Blastoma (PPB) is a rare lung cancer in children. This study looks at how well surgery with or without chemotherapy works for treating it. Chemotherapy is a treatment using drugs to kill cancer cells. Type I PPB patients may receive surgery alone or with chemotherapy, while Types II and III PPB patients may receive additional drugs like topotecan, which helps stop cancer cells from growing.
Children under 21 with newly diagnosed PPB can join the study. Some will get chemotherapy drugs like vincristine, ifosfamide, and dactinomycin, which work by damaging the DNA of cancer cells to kill them. Topotecan is a special drug that may further shrink the tumor or help prevent it from spreading.
- Study length: up to 5 years with regular follow-ups.
- Participants may receive surgery, chemotherapy, or observation depending on their PPB type.
- New treatment methods are being explored to find better ways to treat PPB.
Participants should not have had previous cancer treatment and must meet certain health criteria. Pregnant individuals are excluded, and participants must agree to use birth control if needed.