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Last update 08 May 2025

Pleuropulmonary Blastoma

Last update 08 May 2025

Basic Info

Synonyms

PLEUROPULMONARY BLASTOMA, PPB, PPB FAMILIAL TUMOR AND DYSPLASIA SYNDROME

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Introduction

A malignant neoplasm affecting the lungs and/or the pleura. Pleuropulmonary blastoma is seen in children. Microscopically, the tumor may show features of chondrosarcoma, leiomyosarcoma, rhabdomyosarcoma, liposarcoma, or undifferentiated sarcoma. In approximately 25% of patients with pleuropulmonary blastoma, there are other lesions or neoplasms that may affect patients or their families, including lung or kidney cysts, and ovarian or testicular neoplasms. Heterozygous germline mutations in DICER1 gene have been identified in families harboring pleuropulmonary blastomas.

Clinical Trials associated with Pleuropulmonary Blastoma

NCT06647953

/ RecruitingPhase 3IIT

Prospective Treatment of Types I, II and III Pleuropulmonary Blastoma (PPB)

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.

Start Date21 Mar 2025

Sponsor / Collaborator

The Children's Oncology Group Foundation, Inc.

ChiCTR1900024756

/ SuspendedNot Applicable

A registry study of pleuropulmonary blastoma and Dicer 1 related conditions

Start Date01 Aug 2019

Sponsor / Collaborator

National Children's Center, Inc.

NCT03681522

/ Unknown statusNot ApplicableIIT

What is the Most Effective Local Anesthesia for Prostate-stained Ultrasonography-guided Biopsy in PPB and PNB?: A Single Center, Prospective, Randomized, Double Arm Study

This study assesses differences in the effects of pelvic plexus block and periprostatic nerve block among various anesthetic methods that reduce the pain caused by prostate biopsy.

Start Date10 Sep 2018

Sponsor / Collaborator

Gangnam Severance Hospital

100 Clinical Results associated with Pleuropulmonary Blastoma

100 Translational Medicine associated with Pleuropulmonary Blastoma

0 Patents (Medical) associated with Pleuropulmonary Blastoma

872

Literatures (Medical) associated with Pleuropulmonary Blastoma

01 May 2025·Cancer

Ovarian juvenile granulosa cell tumor: A report from the International Ovarian and Testicular Stromal Tumor and International Pleuropulmonary Blastoma/DICER1 Registries

Article

Author: Schneider, Dominik T. ; Levy, Carolyn Fein ; Kamihara, Junne ; Watson, Dave ; Devins, Kyle M. ; Stall, Jennifer N. ; Young, Robert H. ; Dehner, Louis P. ; Hill, D. Ashley ; Harris, Anne K. ; Tadavarthy, Anil K. ; Nelson, Alexander T. ; Stering, Allen ; Mallinger, Paige H. R. ; Lagmay, Joanne ; Chen, Kenneth S. ; Foresto, Steve ; Herzog, Cynthia E. ; Messinger, Yoav H. ; Schultz, Kris Ann P. ; Billmire, Deborah F. ; Frazier, A. Lindsay ; Snyder, Stacy L.

AbstractBackgroundOvarian juvenile granulosa cell tumors (juvGCT) are rare sex cord‐stromal tumors that occur primarily in children and adolescents. This study summarizes the clinical presentation and outcomes of patients with juvGCT.MethodsPatients were enrolled in the International Ovarian and Testicular Stromal Tumor and/or International Pleuropulmonary Blastoma/DICER1 Registries. Available medical records were abstracted, and pathology was centrally reviewed. Surgical staging was classified using the 2014 International Federation of Gynecology and Obstetrics (FIGO) criteria.ResultsIn total, 70 patients with juvGCT enrolled and were diagnosed between 2001 and 2024; most patients (81%, 57 of 70) presented with FIGO stage I disease. Adjuvant chemotherapy was given in 30% (21 of 70); all regimens were platinum‐based. Three‐year event‐free survival among patients with stage IA tumors was 80.2% (95% confidence interval [CI], 62.4%–100.0%), IC1 was 87.4 (95% CI, 72.4%–100.0%), IC2‐IC3 was 63.6% (95% CI, 40.7%–99.5%), and II‐IV was 48% (95% CI, 24.6%–93.8%). Of the patients with recurrent juvGCT with known mitotic index (MI), all had MI greater than 19 mitoses per 10 high power fields (HPF) at diagnosis.ConclusionOutcomes were worse for patients with FIGO stage ≥IC2 disease and for tumors with >19 mitoses per 10 HPF. Given the prognostic significance of MI, the authors strongly recommend the assessment of MI for all juvGCTs. More information about tumor biology is critical to identify which patients may benefit from adjuvant chemotherapy and to facilitate the development of novel therapies.

01 May 2025·Pediatric Blood & Cancer

A Unique Case of Recurrence After Adjuvant Chemotherapy in Pleuropulmonary Blastoma (PPB) Type I With Transformation to PPB Type III

Letter

Author: Gegeliya, Nina V. ; Druy, Alexander E. ; Usman, Natalia Yu. ; Sidorov, Ilya V. ; Tverdov, Ivan V. ; Andreeva, Natalia A. ; Schultz, Kris Ann P. ; Konovalov, Dmitry M. ; Kachanov, Denis Yu. ; Abasov, Ruslan Kh. ; Lemeshev, Mikhail A. ; Shamanskaya, Tatyana V.

01 Apr 2025·Gastroenterología y Hepatología

Periendoscopic management of antithrombotic drugs: Is cold snare polypectomy the answer?

Review

Author: Argumánez, Victor ; Plumé, Gema ; Bustamante-Balén, Marco

Patients undergoing colonoscopy are increasingly taking antithrombotic medication. These patients, who are generally older, also have a higher prevalence of colon polyps. Therefore, it is general practice to modify or discontinue antithrombotic treatment before colonoscopy, to reduce the risk of post-polypectomy bleeding (PPB). However, this modification increases the risk of thrombotic events. Currently, the main clinical guidelines recommend cold-snare resection for polyps smaller than 10mm because of its better safety profile, especially in reducing post-polypectomy bleeding. This reduced PPB rate could open the door to maintaining antithrombotic drugs in patients undergoing colonoscopy. This review aims to evaluate the evidence available so far that may support a modification of current guidelines on the management of antithrombotic drugs in the periendoscopic period.

News (Medical) associated with Pleuropulmonary Blastoma

18 Apr 2023

·www.prnewswire.com

Star Gala inspires record-breaking donations to support life-saving resources for Children's Minnesota cancer and blood disorders program

MINNEAPOLIS and ST. PAUL, Minn., April 17, 2023 /PRNewswire/ -- Children's Minnesota announced today that a record-breaking $2.36 million was raised at this year's Star Gala – one of the biggest nights of the year for the philanthropic community in the Twin Cities. The 31st annual Star Gala benefited the Children's Minnesota cancer and blood disorders program, the largest program of its kind in the Upper Midwest. The event, hosted by KARE 11's Belinda Jensen, brought business leaders, philanthropists and community members to The Depot in Minneapolis on Saturday, April 15 to raise vital funds that will be invested in life-saving resources, wrap-around services for patient families, and cutting-edge cancer and blood disorders research. "Our program offers advanced, individualized care for our patients because we know that not every cancer and blood disorder diagnosis is the same. We also have the largest clinical research staff in the region, and we participate in over 100 research studies, meaning our patients have access to innovative treatments before they're widely available," said Dr. Sue Kearney, medical director of the cancer and blood disorders program. "We are grateful for the generosity of our community. Their support of our program allows us to continue advancing the treatment of cancer and blood disorders and provide the highly specialized care that can get kids on the road to recovery." To support Children's Minnesota, visit ChildrensMN.org/StarGala. The cancer and blood disorders program at Children's Minnesota cares for more than 3,500 patients from infants to young adults each year, including more than 80 percent of Minnesota children with sickle cell disease or hemoglobinopathies and other hemophilia diagnoses. Children's Minnesota is a part of the Therapeutic Advances in Childhood Leukemia & Lymphoma (TACL) consortium, which offers eligible patients with recurrent leukemias and lymphomas access to early studies of new drugs. The program is also a member of the Pacific Pediatric Neuro-Oncology Consortium (PNOC), which is made up of 18 children's hospitals dedicated to improving the outcomes for children with brain and spinal cord tumors. In addition, Children's Minnesota is home to the International Ovarian and Testicular Stromal Tumor Registry and International Pleuropulmonary Blastoma/DICER1 Registry. The registries collect clinical and biological information about these rare tumors in order to improve treatment and screening. The Star Gala was made possible by presenting sponsor APi Group. Other top sponsors at this year's event include: Baird Private Wealth Management and Great Clips. About Children's Minnesota Children's Minnesota is one of the largest pediatric health systems in the United States and the only health system in Minnesota to provide care exclusively to children, from before birth through young adulthood. An independent and not-for-profit system since 1924, Children's Minnesota serves kids throughout the Upper Midwest at two free-standing hospitals, nine primary care clinics, multiple specialty clinics and seven rehabilitation sites. As The Kids ExpertsTM, Children's Minnesota is regularly ranked by U.S. News & World Report as a top children's hospital. Find us on Facebook @childrensminnesota or on Twitter and Instagram @childrensmn. Please visit childrensMN.org. SOURCE Children's Minnesota

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