Actively Recruiting
Testing a Standardized Approach to Surgery and Chemotherapy for Type I Pleuropulmonary Blastoma or the Addition of an Anti-cancer Drug, Topotecan, to the Usual Treatment for Types II and III Pleuropulmonary Blastoma
Led by Children's Oncology Group · Updated on 2026-05-05
110
Participants Needed
82
Research Sites
210 weeks
Total Duration
On this page
AI-Summary
What this Trial Is About
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.
CONDITIONS
Official Title
Testing a Standardized Approach to Surgery and Chemotherapy for Type I Pleuropulmonary Blastoma or the Addition of an Anti-cancer Drug, Topotecan, to the Usual Treatment for Types II and III Pleuropulmonary Blastoma
Who Can Participate
Eligibility Criteria
You may qualify if you...
- 21 years of age or younger
- Newly diagnosed with pleuropulmonary blastoma (PPB), including Type I, Ir, II, or III diagnosed within 60 days before enrollment
- Patients with known germline DICER1 variant or mosaicism with a large, solid unresectable thoracic mass characteristic of Type II or III PPB may be eligible without biopsy if unsafe
- For Types II or III PPB, serum creatinine, bilirubin, and liver enzyme levels must meet specified age and sex-based limits
- Heart function must be adequate as shown by echocardiogram or radionuclide angiogram
- HIV-infected patients on effective anti-retroviral therapy with undetectable viral load within 6 months are eligible if not on strong CYP3A4 inhibitors or inducers
You will not qualify if you...
- Prior chemotherapy directed at pleuropulmonary blastoma (PPB)
- Known Charcot-Marie-Tooth disease
- Pregnant females
- Lactating females planning to breastfeed
- Sexually active patients of reproductive potential not agreeing to use effective contraception during the study
- Failure to sign informed consent or meet regulatory requirements for human studies
AI-Screening
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Trial Site Locations
Total: 82 locations
1
Children's Hospital of Alabama
Birmingham, Alabama, United States, 35233
Actively Recruiting
2
Phoenix Childrens Hospital
Phoenix, Arizona, United States, 85016
Actively Recruiting
3
Arkansas Children's Hospital
Little Rock, Arkansas, United States, 72202-3591
Actively Recruiting
4
Loma Linda University Medical Center
Loma Linda, California, United States, 92354
Actively Recruiting
5
Children's Hospital Los Angeles
Los Angeles, California, United States, 90027
Actively Recruiting
6
Valley Children's Hospital
Madera, California, United States, 93636
Actively Recruiting
7
UCSF Benioff Children's Hospital Oakland
Oakland, California, United States, 94609
Actively Recruiting
8
Kaiser Permanente-Oakland
Oakland, California, United States, 94611
Actively Recruiting
9
UCSF Medical Center-Mission Bay
San Francisco, California, United States, 94158
Actively Recruiting
10
Children's Hospital Colorado
Aurora, Colorado, United States, 80045
Actively Recruiting
11
Rocky Mountain Hospital for Children-Presbyterian Saint Luke's Medical Center
Denver, Colorado, United States, 80218
Actively Recruiting
12
Alfred I duPont Hospital for Children
Wilmington, Delaware, United States, 19803
Actively Recruiting
13
Children's National Medical Center
Washington D.C., District of Columbia, United States, 20010
Actively Recruiting
14
Golisano Children's Hospital of Southwest Florida
Fort Myers, Florida, United States, 33908
Actively Recruiting
15
Nemours Children's Clinic-Jacksonville
Jacksonville, Florida, United States, 32207
Actively Recruiting
16
Nicklaus Children's Hospital
Miami, Florida, United States, 33155
Actively Recruiting
17
Arnold Palmer Hospital for Children
Orlando, Florida, United States, 32806
Actively Recruiting
18
Nemours Children's Hospital
Orlando, Florida, United States, 32827
Actively Recruiting
19
Nemours Children's Clinic - Pensacola
Pensacola, Florida, United States, 32504
Actively Recruiting
20
Saint Joseph's Hospital/Children's Hospital-Tampa
Tampa, Florida, United States, 33607
Actively Recruiting
21
Children's Healthcare of Atlanta - Arthur M Blank Hospital
Atlanta, Georgia, United States, 30329
Actively Recruiting
22
Lurie Children's Hospital-Chicago
Chicago, Illinois, United States, 60611
Actively Recruiting
23
University of Chicago Comprehensive Cancer Center
Chicago, Illinois, United States, 60637
Actively Recruiting
24
Riley Hospital for Children
Indianapolis, Indiana, United States, 46202
Actively Recruiting
25
University of Iowa/Holden Comprehensive Cancer Center
Iowa City, Iowa, United States, 52242
Actively Recruiting
26
University of Kentucky/Markey Cancer Center
Lexington, Kentucky, United States, 40536
Not Yet Recruiting
27
Norton Children's Hospital
Louisville, Kentucky, United States, 40202
Actively Recruiting
28
Children's Hospital New Orleans
New Orleans, Louisiana, United States, 70118
Actively Recruiting
29
Johns Hopkins University/Sidney Kimmel Cancer Center
Baltimore, Maryland, United States, 21287
Actively Recruiting
30
Dana-Farber Cancer Institute
Boston, Massachusetts, United States, 02215
Actively Recruiting
31
C S Mott Children's Hospital
Ann Arbor, Michigan, United States, 48109
Actively Recruiting
32
Children's Hospital of Michigan
Detroit, Michigan, United States, 48201
Actively Recruiting
33
Corewell Health Grand Rapids Hospitals - Helen DeVos Children's Hospital
Grand Rapids, Michigan, United States, 49503
Actively Recruiting
34
Bronson Methodist Hospital
Kalamazoo, Michigan, United States, 49007
Actively Recruiting
35
Children's Hospitals and Clinics of Minnesota - Minneapolis
Minneapolis, Minnesota, United States, 55404
Actively Recruiting
36
Mayo Clinic in Rochester
Rochester, Minnesota, United States, 55905
Actively Recruiting
37
University of Mississippi Medical Center
Jackson, Mississippi, United States, 39216
Actively Recruiting
38
Children's Mercy Hospitals and Clinics
Kansas City, Missouri, United States, 64108
Actively Recruiting
39
Washington University School of Medicine
St Louis, Missouri, United States, 63110
Actively Recruiting
40
Children's Hospital and Medical Center of Omaha
Omaha, Nebraska, United States, 68114
Actively Recruiting
41
University of Nebraska Medical Center
Omaha, Nebraska, United States, 68198
Actively Recruiting
42
Rutgers Cancer Institute of New Jersey-Robert Wood Johnson University Hospital
New Brunswick, New Jersey, United States, 08903
Actively Recruiting
43
Saint Joseph's Regional Medical Center
Paterson, New Jersey, United States, 07503
Actively Recruiting
44
Albany Medical Center
Albany, New York, United States, 12208
Actively Recruiting
45
The Steven and Alexandra Cohen Children's Medical Center of New York
New Hyde Park, New York, United States, 11040
Actively Recruiting
46
Montefiore Medical Center - Moses Campus
The Bronx, New York, United States, 10467
Actively Recruiting
47
New York Medical College
Valhalla, New York, United States, 10595
Actively Recruiting
48
UNC Lineberger Comprehensive Cancer Center
Chapel Hill, North Carolina, United States, 27599
Actively Recruiting
49
Duke University Medical Center
Durham, North Carolina, United States, 27710
Actively Recruiting
50
Sanford Broadway Medical Center
Fargo, North Dakota, United States, 58122
Actively Recruiting
51
Cincinnati Children's Hospital Medical Center
Cincinnati, Ohio, United States, 45229
Actively Recruiting
52
Rainbow Babies and Childrens Hospital
Cleveland, Ohio, United States, 44106
Actively Recruiting
53
Nationwide Children's Hospital
Columbus, Ohio, United States, 43205
Actively Recruiting
54
Dayton Children's Hospital
Dayton, Ohio, United States, 45404
Actively Recruiting
55
University of Oklahoma Health Sciences Center
Oklahoma City, Oklahoma, United States, 73104
Actively Recruiting
56
Oregon Health and Science University
Portland, Oregon, United States, 97239
Not Yet Recruiting
57
Children's Hospital of Philadelphia
Philadelphia, Pennsylvania, United States, 19104
Actively Recruiting
58
Children's Hospital of Pittsburgh of UPMC
Pittsburgh, Pennsylvania, United States, 15224
Actively Recruiting
59
BI-LO Charities Children's Cancer Center
Greenville, South Carolina, United States, 29605
Actively Recruiting
60
Sanford USD Medical Center - Sioux Falls
Sioux Falls, South Dakota, United States, 57117-5134
Actively Recruiting
61
Saint Jude Children's Research Hospital
Memphis, Tennessee, United States, 38105
Actively Recruiting
62
The Children's Hospital at TriStar Centennial
Nashville, Tennessee, United States, 37203
Actively Recruiting
63
Vanderbilt University/Ingram Cancer Center
Nashville, Tennessee, United States, 37232
Actively Recruiting
64
Dell Children's Medical Center of Central Texas
Austin, Texas, United States, 78723
Actively Recruiting
65
UT Southwestern/Simmons Cancer Center-Dallas
Dallas, Texas, United States, 75390
Actively Recruiting
66
Cook Children's Medical Center
Fort Worth, Texas, United States, 76104
Actively Recruiting
67
Covenant Children's Hospital
Lubbock, Texas, United States, 79410
Actively Recruiting
68
Children's Hospital of San Antonio
San Antonio, Texas, United States, 78207
Actively Recruiting
69
Methodist Children's Hospital of South Texas
San Antonio, Texas, United States, 78229
Actively Recruiting
70
Primary Children's Hospital
Salt Lake City, Utah, United States, 84113
Actively Recruiting
71
University of Virginia Cancer Center
Charlottesville, Virginia, United States, 22908
Actively Recruiting
72
Children's Hospital of The King's Daughters
Norfolk, Virginia, United States, 23507
Actively Recruiting
73
Seattle Children's Hospital
Seattle, Washington, United States, 98105
Not Yet Recruiting
74
Providence Sacred Heart Medical Center and Children's Hospital
Spokane, Washington, United States, 99204
Actively Recruiting
75
Saint Vincent Hospital Cancer Center Green Bay
Green Bay, Wisconsin, United States, 54301
Actively Recruiting
76
University of Wisconsin Carbone Cancer Center - University Hospital
Madison, Wisconsin, United States, 53792
Actively Recruiting
77
IWK Health Centre
Halifax, Nova Scotia, Canada, B3K 6R8
Actively Recruiting
78
Hospital for Sick Children
Toronto, Ontario, Canada, M5G 1X8
Actively Recruiting
79
The Montreal Children's Hospital of the MUHC
Montreal, Quebec, Canada, H3H 1P3
Actively Recruiting
80
Centre Hospitalier Universitaire Sainte-Justine
Montreal, Quebec, Canada, H3T 1C5
Actively Recruiting
81
Centre Hospitalier Universitaire de Sherbrooke-Fleurimont
Sherbrooke, Quebec, Canada, J1H 5N4
Actively Recruiting
82
CHU de Quebec-Centre Hospitalier de l'Universite Laval (CHUL)
Québec, Canada, G1V 4G2
Actively Recruiting
How is the study designed?
Study Type
INTERVENTIONAL
Masking
NONE
Allocation
NON_RANDOMIZED
Model
SEQUENTIAL
Primary Purpose
TREATMENT
Number of Arms
4
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