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Pleuropulmonary BlastomaChildren's healthProcedureDrugBiologicalPhase 3

What this study is

This phase III trial tests how well surgery plus chemotherapy compared to surgery alone works in treating patients with type I pleuropulmonary blastoma (PPB), and tests how well surgery plus standard chemotherapy with the addition of topotecan works compared to surgery plus standard chemotherapy alone in treating patients with type II and III PPB.

Historically, most children with type I PPB had surgery and approximately 40% of children with type I PPB received chemotherapy following their surgery, usually for 22-42 weeks. There has not been a consistent standard for which children with type I PPB receive chemotherapy after surgery. For patients whose tumor has been removed completely with surgery, observation without chemotherapy may work as well as giving chemotherapy after surgery in preventing a return of the PPB tumor.

The standard chemotherapy for patients with types II or III PPB in the United States is four cycles of IVADo (ifosfamide, vincristine, dactinomycin, and doxorubicin) followed by 8 cycles of IVA (ifosfamide, vincristine and dactinomycin). Ifosfamide is in a class of medications called alkylating agents. It works by slowing or stopping the growth of tumor cells in the body. Vincristine is in a class of medications called vinca alkaloids. It works by stopping tumor cells from growing and dividing and may kill them. Dactinomycin is a type of antibiotic that is only used in cancer chemotherapy (antineoplastic antibiotic). It works by damaging the cell's deoxyribonucleic acid (DNA) and may kill tumor cells. Doxorubicin is in a class of medications called anthracyclines. Doxorubicin damages the cell's DNA and may kill tumor cells. It also blocks a certain enzyme needed for cell division and DNA repair. Topotecan is in a class of medications called topoisomerase I inhibitors. It works by interfering with tumor cell DNA which kills them. Giving topotecan in addition to standard IVADo and IVA chemotherapy regimens may shrink the cancer as well as or better than the standard therapy or could decrease the chance the tumor spreads while causing fewer side effects.

What's being tested

  • Biospecimen Collection

    Intervention · PROCEDURE

    Undergo collection of blood samples

  • Biospecimen Collection

    Intervention · PROCEDURE

    Tumor tissue is collected and centrally reviewed by a study pathologist

  • Bone Scan

    Intervention · PROCEDURE

    Undergo bone scan

  • Computed Tomography

    Intervention · PROCEDURE

    Undergo CT

  • Cyclophosphamide

    Intervention · DRUG

  • Dactinomycin

    Intervention · BIOLOGICAL

  • Dexrazoxane

    Intervention · DRUG

  • Doxorubicin

    Intervention · DRUG

  • Echocardiography Test

    Intervention · PROCEDURE

    Undergo ECHO

  • Ifosfamide

    Intervention · DRUG

  • Magnetic Resonance Imaging

    Intervention · PROCEDURE

    Undergo MRI

  • Multigated Acquisition Scan

    Intervention · PROCEDURE

    Undergo MUGA

  • Patient Observation

    Intervention · OTHER

    Undergo observation

  • Positron Emission Tomography

    Intervention · PROCEDURE

    Undergo PET

  • Topotecan

    Intervention · DRUG

  • Ultrasound Imaging

    Intervention · PROCEDURE

    Undergo ultrasound

  • Vincristine

    Intervention · DRUG

All of Cyclophosphamide, Dactinomycin, Dexrazoxane, Doxorubicin, Ifosfamide, Topotecan, Vincristine

Given IV

Study details

Official title
Prospective Treatment of Types I, II and III Pleuropulmonary Blastoma (PPB)
Sponsor
Children's Oncology Group
Conditions
Pleuropulmonary Blastoma
Enrolling
110 people
Starts
2025-03-21
Est. completion
2029-03-31
Design
Open-label, non-randomized, sequential groups, Phase 3
Record
View on ClinicalTrials.gov ↗