Related Experiment Video
Updated: Oct 21, 2025
Murine Model of Leukemia Relapse to Induction Chemotherapy for Acute Lymphoblastic Leukemia
Published on: October 17, 2025
Children with progressive and relapsed pleuropulmonary blastoma: A European collaborative analysis
Monika Sparber-Sauer1, Arianna Tagarelli2, Guido Seitz3
1Klinikum der Landeshauptstadt Stuttgart gKAäR, Olgahospital, Stuttgart Cancer Center, Zentrum für Kinder-, Jugend- und Frauenmedizin, Pädiatrie 5 (Pädiatrische Onkologie, Hämatologie, Immunologie), Stuttgart, Germany.
Insights
Pleuropulmonary blastoma (PPB) type II/III in children with progressive disease (PD) is fatal. While rare, some children with relapsed disease (RD) can achieve remission with multimodal treatment, highlighting the need for novel therapies for progressive PPB.
Area of Science:
- Pediatric Oncology
- Thoracic Surgery
- Medical Genetics
Background:
- Pleuropulmonary blastoma (PPB) type II/III presents a significant challenge in pediatric oncology.
- Children diagnosed with progressive disease (PD) or relapsed disease (RD) of PPB type II/III historically face a poor prognosis.
Purpose of the Study:
- To analyze outcomes for children with progressive or relapsed pleuropulmonary blastoma (PPB) type II/III.
- To evaluate the efficacy of current treatment modalities for advanced PPB.
- To identify factors influencing survival in pediatric PPB patients.
Main Methods:
- Retrospective analysis of 35 pediatric patients diagnosed with PPB type II/III and either PD or RD.
- Data collected from national and European databases and trials between 2000 and 2018.
- Evaluation of treatment strategies including surgery, chemotherapy (CHT), and radiotherapy (RT), and their impact on survival outcomes.
Main Results:
- All 9 patients with progressive disease (PD) died. Median age at progression was 3.9 years.
- For 26 patients with relapsed disease (RD), 5-year event-free survival (EFS) and overall survival (OS) were both 37%.
- Local therapies (surgery and RT) showed a favorable impact on overall survival (p=0.03 and p=0.02, respectively).
Conclusions:
- Achieving a cure for relapsed pleuropulmonary blastoma (PPB) type II/III is possible but uncommon, even with multimodal treatment.
- Progressive PPB remains a fatal condition, underscoring the urgent need for the development of new therapeutic strategies.
- Further research into innovative treatments is critical for improving outcomes in pediatric patients with advanced PPB.
Background:
Children with progressive (PD) or relapsed disease (RD) of pleuropulmonary blastoma (PPB) type II/III are known to have a very poor outcome.
Methods:
A retrospective review of children registered in national and European databases and trials (2000-2018) with diagnosis of PPB type II/III and PD or RD was performed.
Results:
A total of 35 patients with PPB were analysed: patients with PD (n = 9) and RD (n = 26). Patients experienced PD at the median age of 3.9 years [range, 0.5-17.8] despite surgery, chemotherapy (CHT, n = 9) and radiotherapy (RT, n = 1) with a median time to progression of 0.58 years [range, 0.02-1.27] from diagnosis. All of them died. Patients suffered from RD at the median age of 4.3 years [1.7-15.1], median delay to relapse 1.03 years [range, 0.03-2.95]. RD occurred locally (n = 12), combined (n = 1) and in metastatic sites (n = 13): central nervous system (n = 11) and unspecified site (n = 2). Patients were treated with salvage CHT (n = 20), surgery (n = 10) ± RT (n = 10). After a median follow-up of 4.2 years [range, 2.1-14.6], a second complete remission (CR) was achieved in nine out of 26 patients. Patients were alive in the second CR (n = 6), in the third CR (n = 1), in partial remission (n = 2) and lost of follow-up (n = 1). Five-year event-free survival (EFS) and overall survival (OS) for patients with RD were both 37% (±19, CI 95%). Local therapy (surgery, RT) had a favourable impact on OS (p = 0.03 and 0.02, respectively).
Conclusions:
Cure of patients with RD of PPB type II/III with multimodal treatment is possible but rare. Progressive PPB is fatal and patients need new treatment options.
More Related Videos
04:04Endobronchial Ultrasound-guided Intratumoral Injection of Cisplatin for the Treatment of Isolated Mediastinal Recurrence of Lung Cancer
Published on: February 12, 2017
08:34Proton Therapy Delivery and Its Clinical Application in Select Solid Tumor Malignancies
Published on: February 6, 2019
Related Concept Videos
02:56Treatment Resistant Cancers
01:29Pulmonary Embolism II: Diagnostic Studies and Interprofessional Care